SlideShare ist ein Scribd-Unternehmen logo
1 von 346
Downloaden Sie, um offline zu lesen
NEUROMUSCULAR
DISORDER
The Neuron
 Defining unit of the nervous system
 Specialized cell of the nervous system
 Consists of a cell body, 5–25 µm in diameter, with branching processes (dendrites) that are
capable of receiving signals from other neuronal terminals
 A finer, longer branch (the axon) carries the action potentials along its length to or from
excitable target organs
 Further signal transmission to the dendrites of another neuron, or neuro-excitable tissue like
muscle, occurs at a synapse where the axon terminal releases a chemical neurotransmitter –
typically acetylcholine
 All motor axons and the larger sensory axons serving touch, pain and
proprioception are covered by a sheath – the neurilemma – and coated with
myelin, a multilayered lipoprotein substance derived from the accompanying
Schwann cells (or oligodendrocytes in the central nervous system).
 Every few millimetres the myelin sheath is
interrupted, leaving short segments of bare axon
called the nodes of Ranvier.
 In these nerves the myelin coating serves as an
insulator, which allows the impulse to be propagated
by electromagnetic conduction from node to node,
much faster than is the case in unmyelinated nerves.
 Consequently, depletion of the myelin sheath causes
slowing – and eventually complete blocking – of
axonal conduction
 Most axons, in particular the small-
diameter fibres carrying crude sensation
and efferent sympathetic fibres, are not
myelinated but wrapped in Schwann cell
cytoplasm.
 Damage to these axons causes unpleasant
or bizarre sensations and abnormal
sudomotor and vasomotor effects.
NERVOUS PATHWAYS
 Anatomically, neurological structures can be divided into the central
nervous system (the CNS, comprising the brain and tracts of the spinal cord)
and the peripheral nervous system (PNS) which includes the cranial and
spinal nerves
 In terms of physiological function, both the CNS and the PNS have a somatic
component and an autonomic component
 The somatic nervous system provides efferent motor and afferent sensory
pathways to and from peripheral parts of the body serving, respectively,
voluntary muscle contraction and sensibility
 The autonomic system controls involuntary reflex and homeostatic activities
of the cardiovascular system, visceral organs and glands. Its two
components, sympathetic and parasympathetic divisions, serve more or less
opposing functions.
CNS and PNS
 Central nervous system consists of the brain and spinal cord
 Peripheral nervous system constitutes the link between the CNS
and structures in the periphery of the body, from which it
receives sensory information and to which it sends controlling
impulses
 T he peripheral nervous system consists of nerves joined to the
brain and spinal cord (cranial and spinal nerves) and their
ramifications within the body
Main nerve pathways
Simplified diagram showing the main neurological pathways to and
from a typical thoracic spinal cord segment. Fibres carrying touch,
sharp pain and temperature impulses (-------) decussate, in some
cases over several spinal segments, and ascend in the contralateral
spinothalamic tracts; those carrying vibration and proprioceptive
impulses (——) enter the ipsilateral posterior columns. Motor neurons
(——) arise in the anterior horn of the grey matter and innervate
ipsilateral muscles.
NEUROMUSCULAR
DISORDER
SOMATIC MOTOR SYSTEM
 Efferent impulses are conducted along axons in the corticospinal or
pyramidal tracts (upper motor neurons – UMN) and along peripheral nerves
from cell bodies in the anterior horn of the spinal cord to striated muscle
fibres (lower motor neurons – LMN)
 The terminal synapses are situated at the neuromuscular junctions.
 Each large a-motor neuron innervates from a few to several hundred muscle
fibres (together forming a motor unit) and stimulates muscle fibre
contraction.
 In large muscles of the lower limb, power is adjusted by recruiting more or
fewer motor units.
 Smaller γ-motor neurons connect to sensors (muscle spindles) that control
proprioceptive feedback from muscle fibres.
SOMATIC MOTOR SYSTEM
SOMATIC SENSORY SYSTEM
 Axons conveying afferent impulses from receptors in the skin and other
peripheral structures enter the dorsal nerve roots, with their cell bodies in
the dorsal root (or cranial nerve) ganglia, and end in synapses within the
central nervous system.
 Myelinated fibres carrying sensory stimuli from touch, pressure, pain and
temperature (exteroceptive sensation) decussate and enter the contralateral
spinothalamic tracts running up the spinal cord to the brain.
 Fibres from sensors in the joints, ligaments, tendons and muscle carrying the
sense of movement and bodily position in space (proprioceptive sensation)
join the ipsilateral posterior columns in the spinal cord.
SOMATIC SENSORY SYSTEM
Neuromuscular disorder
REFLEX ACTIVITY AND TONE
 Sudden stretching of a muscle (e.g. by tapping sharply over the tendon)
induces an involuntary muscle contraction – the stretch reflex
 The sharp change in muscle fibre length is detected by the muscle spindle;
the impulse is transmitted rapidly along myelinated afferent (sensory)
neurons which synapse directly with the corresponding segmental α-motor
neurons in the spinal cord, triggering efferent signals which stimulate the
muscle to contract.
Neuromuscular disorder
 Segmental reflex activity is normally regulated by motor impulses passing
from the brain down the spinal cord.
 Interruption of the UMN pathways results in undamped reflex muscle
contraction (clinically hyperactive tendon reflexes) and spastic paralysis.
 Damage to either afferent or efferent neurons in the reflex arc causes
hypotonia; interruption of the LMN pathway results in flaccid LMN paralysis.
AUTONOMIC SYSTEM
 The autonomic system is involved with the regulation of involuntary
activities of cardiac muscle and smooth (unstriated) muscle of the lungs,
gastrointestinal tract, kidneys, bladder, genital organs, sweat glands and
small blood vessels, with afferent (sensory) and efferent (motor) pathways
constituting a continuously active reflex arc (though there is also some input
from higher centres).
 In addition afferent fibres also convey visceral pain sensation.
 Preganglionic sympathetic neurons leave the spinal cord with the ventral nerve roots at all
levels from T1 to L1, enter the paravertebral sympathetic chain of ganglia and synapse with
postganglionic neurons that spread out to all parts of the body; they may also run up or down
the sympathetic chain to synapse in other ganglia or pass on to become splanchnic nerves
 Important functions are the reflex control of heart rate, blood flow and sweating, as well as
other responses associated with conditions of ‘fight and flight’.
Neuromuscular disorder
 Parasympathetic neurons leave the CNS (from the brain-stem) with cranial
nerves III, VII, IX, X and with the nerve roots of S2, 3 and 4 to reach ganglia
where they synapse with postganglionic neurons close to their target organs.
Neuromuscular disorder
Peripheral nerves
 Peripheral nerves are bundles of axons
conducting efferent (motor) impulses from cells
in the anterior horn of the spinal cord to the
muscles, and afferent (sensory) impulses from
peripheral receptors via cells in the posterior
root ganglia to the cord.
 They also convey sudomotor and vasomotor
fibres from ganglion cells in the sympathetic
chain.
Peripheral Nerve
Structure
Neuromuscular disorder
SKELETAL MUSCLE
 Each skeletal muscle belly, held within a connective tissue epimysium,
consists of thousands of muscle fibres, separated into bundles (or fascicles).
 Each fascicle is surrounded by a flimsy perimysium which envelops anything
up to about 100 muscle fibres; large muscles concerned with mass
movement, like the glutei or quadriceps, have a large number of fibres in
each fascicle, while muscles used for precision movements (like those of the
hand) have a much smaller number in each bundle.
 The muscle fibre is the important unit of all striated muscle. Lying in a barely
discernable connective tissue cover, or endomysium, it is in actuality a
single cell with a cell membrane (the sarcolemma), a type of cytoplasm (or
sarcoplasm), mitochondria and many thousands of nuclei; its diameter is
about 10 µm at birth and 60–80 µm in mature adults
 The α-motor neuron and the group of muscle fibres it supplies constitute a
single motor unit; the number of muscle fibres in the unit may be less than
five in muscles concerned with fine manipulatory movements or more than
100 in those employed in gross power movements.
SKELETAL MUSCLE
Muscle Fibre
 Muscle fibres are also of different types, which can be distinguished by
histochemical staining.
 Type I fibres contract slowly and are not easily fatigued; their prime
function is postural control.
 Type II fibres are fast contracting but they fatigue rapidly; hence they are
ideally suited to intense activities of short duration.
 All muscles consist of a mixture of fibre types, the balance depending on
anatomical site, basic muscle function, degree of training, genetic
disposition and response to previous injury or illness.
 Long-distance runners have a greater proportion of type I fibres than the
average in age- and sex-matched individuals
NEUROMUSCULAR
DISORDER
Muscle Contraction
 Muscle contraction is a complex activity. Individual myofibrils respond to
electrical stimuli in much the same way as do motor neurons. However,
muscle fibres, and the muscle as a whole, are activated by overlap and
summation of contractile responses.
 When the fibres contract, internal tension in the muscle increases.
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
 In isometric contraction there is increased tension without actual shortening
of the muscle or movement of the joint controlled by that muscle.
 In isotonic contraction the muscle shortens and moves the joint, but tension
within the muscle fibres remains constant.
Muscle Tone
 Muscle tone is the state of tension in a resting muscle when it is passively
stretched; characteristically tone is increased in upper motor neuron (UMN)
lesions (spastic paralysis) and decreased in lower motor neuron (LMN) lesions
(flaccid paralysis).
Muscle contracture
 Muscle contracture (as distinct from contraction) is the adaptive change
which occurs when a normally innervated muscle is held immobile in a
shortened position for some length of time. If a joint is allowed to be held
flexed for a long time, it may be impossible to straighten it passively without
injuring the muscle.
 Active exercise will eventually overcome the muscle contracture, unless the
muscle has been permanently damaged.
Muscle Wasting
 follows either disuse or denervation; in the former, the fibres are intact but
thinner; in the latter, they degenerate and are replaced by fibrous tissue or
fat
Muscle Fasciculation
 Muscle fasciculation – or muscle twitch – is a local involuntary muscle
contraction of a small bundle of muscle fibres.
 It is usually benign but can be due to motor neuron disease or dysfunction.
CLINICAL ASSESSMENT
History
Age  important
 Arthrogryphosis & spina bifida  at birth
 Cerebral palsy -> later in childhood
 Poliomyelitis  childhood  may be seen in any age
 Spinal cord lesions & peripheral neuropathies  common in
adults
 Orthopaedic surgeon  mainly with residual effects of
neurological disease  may require diagnosis & treatment
throughout life
Muscle weakness :
UMN, LMN or muscle disorders
Type of weakness, distribution, rate of onset 
diagnosis
Numbness & paraesthesiae :
May be main complaints
Important to establish their exact distribution 
localize lesion accurately
Rate of onset & relationship to posture  cause
Deformity
 Common complaint in long-standing disorders
 From muscle imbalance  hand in hand with other symptoms
 Minor degrees of weakness in 1 muscle group may unnoticed 
deformity appears so insidiously  may escape detection  eg.
claw toes, scoliosis
Other Features
 Headache
 Dizziness
 Loss of balance
 Change in visual acuity / hearing
 Disorder of speech
 Loss of bladder / bowel control
Examination
 Complete neurological assessment
 The back  skin changes, local deformities, mobility
Patient’s mental state Muscle tone & power
Natural posture Reflexes
Gait Skin changes
Sense of balance Various modes of sensibility &
autonomic functions (eg. sphincter
control)
Involuntary movements Peripheral blood flow
Muscle wasting Sweating
Grading Muscle Power
 Repetition  progress to be recorded
0 Total paralysis
1 Barely detectable contracture
2 Not enough power to act against gravity
3 Strong enough to act against gravity
4 Still stronger but less than normal
5 Full power
Neuromuscular disorder
Neuromuscular disorder
Dermatomes supplied
by spinal nerve roots
GAIT and POSTURE
 A single gait cycle consists of a stance phase (60 per cent) and a swing phase
(40 per cent) and each full cycle represents the stride length
 Dystonia – This term refers to abnormal posturing (focal or generalized) that
may affect any part of the body and is often aggravated when the patient is
concentrating on a particular motor task such as walking
Gait cycle
Antalgic gait
Spastic gait
Drop Foot Gait
High-Stepping gait
Waddling (Trendelenburg) gait
Ataxic gait
Motor Power and Tone
GRADE DESCRIPTION
0 No muscle action. Total paralysis
1 Minimal muscle contraction
2 Power insufficient to overcome gravity
3 Anti-gravity muscle power
4 Less than full power
5 Full power
Weakness
 Monoplegia
 Indicative for lower motor neuron defect
 Movement affected on clinical test will suggest the anatomical location
 Hemiparesis
 Weakness either the right or left side
 Pathology between cerebral cortex and cervical segment of spinal cord
 Upper Motor Neuron type (spastic)
 Complete loss of power: hemiplegia
Weakness
 Diplegia
 Both upper limb or both lower limb
 Can be either UMN or LMN disorder
 Quadriplegia
 All four limb affected
Deformity
 Unbalanced paralysis
 One group of muscle is too weak to balance the pull of the antagonis
 Balanced paralysis
 The joint assumed that the position imposed on it by gravity and it may feel floppy
or flail
Sensation
Imaging
 Plain X-ray
 Routine for all disorders
 Fracture and dislocation
 CT-Scan
 Reveal relation between bone fragment to nerve structure
 MRI
NEUROPHYSIOLOGICAL STUDIES
Motor Nerve Conduction
 Stimulate electrically at an easy
subcutaneous site until it propagates an
action potensial on target muscle
Measurement:
 Latency
 It takes in ms (millisecond)
 Time for impulse to reach the muscle
 Amplitude of the Compound Muscle Action Potential (CMAP)
 In mV (millivolts)
 Magnitude of the response
 Nerve Conduction Velocity
 Measure the distance from stimulating electrode to the recording elecctrode, and divide by the
latency
 ms  time for impulse to reach muscle  latency
 mV  magnitude of response  amplitude of the evoked
compound muscle action potential (CMAP)
 By measuring the distance from the stimulating electrode to
recording electrode, and setting against latency  nerve
conduction velocity (NCV)  metres/second
 In practice, more useful & accurate to stimulate the nerve at 2
points  distal & proximal site, and subtract distal latency from
proximal latency to obtain a truer measurement for intervening
segment of nerve
 To measure NCV of median nerve in carpal tunnel  stimulating
electrode first distal to carpal tunnel & then in upper forearm
 Amplitude  proportional to number of motor units stimulated : if
patient has lost ½ of nerve fibres in peripheral nerves
(compression, trauma, vascular insufficiency), size of elicited CMAP
will be reduced by ±50% compared to contralateral normal limb
 CMAP on proximal stimulation smaller than distal stimulation 
conduction block  a feature of a potentially recoverable
neuropraxic lesion
Conduction slowing of
uniform degree along the
whole length of nerve 
demyelinating neuropathy
 Charcot – Marie – Tooth
syndrome
Sensory Nerve Conduction
In a similar manner, sensory nerve action potential
(SNAP) may be recorded by stimulating a suitable
subcutaneous sensory nerve & recording with
surface electrodes on the skin over a measured
distance along the same sensory nerve  from
index & middle fingers of median nerve
SNAP is much smaller in amplitude than CMAP 
microvolts
Clinical nerve conduction studies estimate
population of large myelinated sensory or motor
nerves
Type C fibres (small myelinated fibres  pain &
temperature)  amplitude below sensitivity of
recording techniques & slowed velocity (5-10
m/sec)  cannot be tested with standard
clinical techniques
NEUROMUSCULAR
DISORDER
Electromyography (EMG)
 Concentric needle electrode (small hypodermic needle) is
inserted into muscle & connected to oscilloscopic screen &
loudspeaker  record electrical discharge of motor units in a
muscle  visual pattern & crackling sounds
 At rest, normal muscle is silent
 Patient slowly contracts  progressive  in number & also
amplitude of motor unit action potentials  recognizable
pattern
Full recruitment pattern usually looks & sounds
like ‘white noise’  so many motor units firing 
both spikes on screen & crackles from speakers
overlap each other  interference pattern
In nerve disorders, muscle may not be silent at rest
  insertional activity
Motor nerve fibre loss / disruption  changes of active
denervation (fibrillation potentials & positive sharp waves) 
denervated muscle fibres firing spontaneously  7-12 days
after axonal disruption
Chronic neuropathy, with
re-sprouting of remaining
viable nerve fibres 
longer re-innervated
motor units with
polyphasic or higher
amplitude profile
Diagnostic Evaluation of The
Patient
 When investigating a specific nerve root syndrome,
nerve conduction & EMG studies are concentrated
in appropriate anatomical territory  findings are
compared to those in other nerve root territories in
the same level as well as the contralateral (usually
asymptomatic) limb
 Mononeuropathy / plexopathy  compare
conduction values (amplitude & velocity) in 1 limb
to those in the other
 Focal entrapment  reduced amplitude on proximal
stimulation compared to distal stimulation 
conduction block or significant focal conduction slowing
 Neurophysiological signs of neuropathic disorder :
  motor or sensory potentials  nonfunctioning
(perhaps transected) nerves
 Loss of sensory responses (SNAP)  disorder distal
to spinal foramen; intact SNAP in hypaesthetic limb
 disease proximal to foramen
 Conduction block  neuropraxic recoverable
injury
 Denervation changes on EMG >10 days after injury
 significant nerve damage & loss of motor nerve
function
 Any recruited volitional motor units in a weak limb
 potential for recovery
 The presence of intact sensory potential is what
distinguishes root & proximal disease from
peripheral entrapment & plexus disease
Intraoperative Neurophysiological
Techniques Spinal Monitoring : somatosensory evoked responses (SSEP)
 Neurophysiological tests are sometimes necessary during
corrective spinal operations  obviate injury to the cord
 EEG  averaging  records from scalp overlying patient’s
sensory parietal cortex  one must average the obtained
responses from at least 100-200 stimuli to differentiate time-
linked evoked response from the background brain EEG
activity
 The important measured parameter is usually the latency of
the response
 Accidental nerve injury during surgery around spinal cord will
produce a delay in the latency or a sudden loss of the evoked
response
 Other intraoperative techniques :
 Nerve or nerve root stimulation  demonstrate conduction
block or slowing or normal continuity of nerve
 Intraoperative EMG
 Cord-to-cord stimulation & cord-to-cortical potential
measurement  reveal intraoperative evidence of spinal
pathway disruption
NEUROMUSCULAR
DISORDER
CEREBRAL PALSY
Examination
A group of disorders result from non-progressive brain
damage during early development  abnormal
movement & posture
2 : 1000 live births  highest in premature babies &
multiple births
Causes : maternal toxaemia, prematurity, perinatal
anoxia, kernicterus, postnatal brain infections / injury
Birth injury  unusual cause
 May also cause damage to other areas of developing brain
 epilepsy, perceptual & behavioural problems, learning
difficulties
 Main consequence  development of neuromuscular
incoordination, dystonia, weakness, spasticity
 Oro-facial motor incoordination  difficult speech &
swallowing, drooling
 None of these defects implies poor intellect
Classification
 Usually according to type of motor disorder, with subdivisions referring to
topographical distribution of clinical signs
Type of Motor Disorder
Spasticity  commonest  damage to pyramidal
system in CNS   muscle tone & hyper-reflexia
Resistance to passive movement may obscure a
basic weakness of affected muscles
Hypotonia  a phase  several years during early
childhood before features of spasticity become
obvious
Athetosis  continuous, involuntary, writhing
movements  damage to extrapyramidal
systems of CNS
Pure athetoid CP  joint contractures are
unusual, muscle tone is not 
Dystonia  may occur with athetosis  more
generalized  in muscle tone & abnormal
positions induced by activity
Ataxia  muscular incoordination during
voluntary movements  due to cerebellar
damage  balance is poor  walks with a
characteristic wide-based gait
Mixed palsy  combination of spasticity &
athetosis  can make results of surgical
intervention unpredictable
Athetosis
Dystonia
Ataxia
 In some types of CP  considerable variability in ‘tone’ & ‘posture’ from day to day / situation to
situation
 If surgical treatment being considered, never based on a single assessment when, due to stress,
child appears to have abnormally high tone & muscle contractures
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
Topographic Distribution
 Hemiplegia  commonest 
spastic palsy on 1 side of body with
upper limb more severely affected
than lower  most can walk &
respond reasonably well to
treatment
 Diplegia  both sides of body with
lower limbs always most severely
affected
Side to side involvement may be
asymmetrical  asymmetric
diplegia, bilateral hemiplegia
Many cases are secondary to prematurity & periventricular leucomalacia on
brain MRI
Intelligence is often normal
Less severely  reasonable mobility
 Total body involvement 
general & often more
severe disorder affecting
all 4 limbs, trunk, neck,
face with varying degrees
of severity
Usually have low IQ, may
have epilepsy, often
unable to walk, poor
treatment response
Monoplegia 
occasionally in an
upper limb, other
areas are involved as
well
True monoplegia  so
unusual  other
diagnoses should be
considered (eg.
Neonatal brachial
plexopathy)
Neuromuscular disorder
Diagnosis in Infancy
 Full-blown clinical picture may take months /
years to develop
 Prenatal toxaemia, haemorrhage, premature
birth, difficult labour, foetal distress,
kernicterus  arouse suspicion
 Neonatal ultrasound scan of head 
intracerebral bleeding
 Early symptoms:
 Difficulty in sucking & swallowing, dribbling at mouth
 Baby feels stiff / wriggles awkwardly
 Apparent that motor milestones are delayed
 Normal : holds up its head at 3 mo, sits up at 6 mo, begins walking at 1 year
Diagnosis in Later Childhood
 Bleck (1987)  7 tests for children over 1 year  idea of
severity & prognosis for walking
 Primitive neck-righting reflex, asymmetrical &
symmetrical tonic neck reflexes, Moro reflex, extensor
thrust response  all disappeared at 1 year
 Retain > 2 primitive reflexes, can’t sit unsupported by 4
yo, can’t walk unaided by 8 yo  unlikely ever to walk
independently
Ideally reviewed by a multidisciplinary team
Gross Motor Function Classification System
(GMFCS)  relative to their age, in terms of
mobility & bases this on their average
function, not the best that they can achieve
on a given occasion  reliable & valid
Neuromuscular disorder
Sitting Posture
 Children with a hypotonic trunk may slump
into a kyphotic posture & others may always
‘fall’ to one side
 In attempting to sit, lower limbs may be
thrust into extension
 May be an obvious scoliosis / pelvis obliquity
Standing Posture
 Typical case of spastic diplegia  stands with hips
flexed, adducted, internally rotated; knees flexed;
feet equinus
 With tight hamstrings, normal lumbar lordosis may
be obliterated & may have difficulty standing
unsupported
 Often attempts to correct 1 deformity may
aggravate another  important to establish which
deformity are primary & compensatory
Many patients show pelvic obliquity &
scoliosis
Asking child to ‘stand tall’, watching their
response often gives some insight into
dynamic nature of posture & muscle
strength, intellectual ability
Balance reactions are often poor
Neuromuscular disorder
Gait
 Observed with & w/o shoes / orthotic supports
 Dystonic, athetoid, ataxic movements may become
more noticeable during walking
 Every opportunity must be taken to observe gait 
differences between ‘normal’ & ‘best behaviour’
walking can be identified
 In hemiplegics, best behaviour walking may
demonstrate a flat foot pattern with heel coming down
most of the time while more normal / representative
pattern will highlight asymmetric flexed knee & toe-
walking pattern
Clinical Gait Analysis
 Each limb must be observed in both stance & swing
phases of gait & in coronal, sagittal & transverse
planes
 Lack of free rotation at hip  trunk has to move
from side to side as each leg swings through & with
adduction  ‘scissoring’ action
 Narrow walking base, when combined with hip &
knee flexion & foot equinus  strong tendency to
fall  helped by cruthces
Computerized Gait Analysis
 Ideally supplements observational gait analysis
 Kinematics (joint & limb segment movement)
 Kinetics (joint moments & powers)
 EMG (identification of phases in which muscles are
firing)
 Pedobarography (foot pressures)
 Metabolic energy analysis (assessment of ‘cost’ of
walking)
• To help clinician distinguish between dynamic & fixed
tightness & in identification of dyskinesia
Neuromuscular Examination
 Typical features of UMN
 Muscle tone, power & ROM at each joint
 Physical signs may vary from day to day / even minute to minute 
emotional state, room temperature
 Takes time
Deformity Assessment
 At each joint & relate it to muscle-tendon length
 Deformity at 1 level may be markedly affected by
position of joints above & below
 Ankle equinus with knee extended often
disappears when knee is flexed  can
differentiate between tightness in soleus &
gastrocnemius muscle
Silfverskiöld Test
 Supine on examination couch
 Knee flexed to a right angle & ankle dorsiflexed  tests soleus
tightness
 Then knee fully extended & ankle dorsiflexion is repeated  tests
gastrocnemius tightness
 Tight hamstrings may limit knee extension more with hips flexed than
when hips extended
 Tight gracilis  hip adduction may be easier in flexion than in
extension
Silfverskiöld Test
Hip abduction is restricted  order x-ray to
look for subluxation of joint
In upper limb, finger flexors may be tight with
wrist extended but if wrist is allowed to flex the
fingers can extend
Children can use these fixed-length reactions to
manipulate their hand & finger function using
‘trick’ movements
 Patient with total body involvement  spinal deformity is
common  scoliosis, often associated with pelvic obliquity
 Kyphosis & lordosis also occur
SENSATION
 Often not entirely normal
 Problems with stereognosis (as well as with perception) 
important factors contributing to upper limb disability
Muscle Contracture
 A degree of muscle contracture is almost
inevitable with all forms of CP  longstanding
spasticity  relative shortening of muscles 
fixed contractures & changes in joint congruity
 Most of the effects seen during period of growth
 After skeletal maturity, changes in muscle-tendon
length & joint contracture much less progressive
Bony Deformity
 Normal bone growth is influenced by muscle pull
 Children with persistent abnormal muscle pull 
failure of normal modelling & new deformities
can develop
 Normal degree of femoral neck anteversion
persists & sometimes even increases with growth
rather than improving  significant external
tibial torsion may also be present
Persistent adduction of hip  valgus of femoral
neck, acetabular dysplasia, subluxation of joint
Flexion deformity of knee  upward
displacement of patella & patello-femoral pain
External tibial torsion  planovalgus deformity
of foot
Structural Scoliosis
Flexible curves are common, but many
become structural  especially likely in
total body involvement
NEUROMUSCULAR
DISORDER
Management
No single ‘blueprint’
Goal Setting
Few patients with total body
involvement will ever talk
Prognosis for walking in spastic diplegia
 Bleck’s criteria & Beals
Priorities for all CP patients are :
Ability to communicate with others
Ability to cope with activities of daily living
(including personal hygiene)
Independent mobility  may mean a
motorized wheelchair rather than walking
Realistic goals for child who from an early age is recognized to be ‘non-walking’ are :
Straight spine with a level pelvis
Located, mobile, painless hip that flex 90o &
extend  comfortable sleeping & participation in
standing / swivel transfers
Knees that mobile enough for sitting, sleeping,
transferring
Plantigrade feet that fit into shoes & rest on
footplates of wheelchair comfortably
Tone Management
Medical treatment 
 anticonvulsants for seizures,
 short-term benzodiazepine for postoperative pain,
 trihexyphenadryl for dystonia
Baclofen
 Agonist gamma-aminobutryic acid (GABA)  inhibits reflex activity
 Oral  doesn’t cross blood-brain barrier well
 Reduces muscle tone / spasticity generally
 Negative effect on head & trunk control  side effects : drowsiness
 its use may be limited
 Intrathecal  via refillable, subcutaneous implanted pump  dose
administered can be titrated according to child’s response
Long-term studies not yet available 
appears most effective in severe spasticity /
dystonia
Not effective in all patients & test doses &
assessment of its benefits required in all
prospective patients
 Dantrolene
Produces weakness w/o much  in spasticity  rarely used in CP
 Analgesic medication
 pain associated with muskuloskeletal problems, constipation, gastro-oesophageal reflux
Botulinum Toxin
 Blocking acetyl choline release at neuromuscular junction
 Injected into ‘spastic’ muscle at (or as near as possible
to) motor end point
 Usual targets : hip adductors, hamstrings, gastrocnemius,
tibialis posterior
 Weakness / paralysis takes a few days to become obvious
 temporary (as new nerve terminals form)  10-12 wk
Not be used on its own
Followed by  physiotherapy input & often an
alteration in orthotic / splinting regimens
Focal treatment for a dynamic muscle
imbalance that is interfering with function 
deformity, pain
More effective in younger children  less likely
to have fixed deformity
Multilevel injections may be required but
overall dose per child must be kept within
safe limits
For postoperative pain & spasm  for
optimal effect, need to be given some days
prior to surgery
Selective Dorsal Rhizotomy
 Division of selected dorsal nerve roots from
L1 to S2 has only recently gained wide
acceptance
  spasticity & rebalance muscle tone 
selectively  input from muscle spindles 
less excitation of anterior horn cells
 Long-term studies not yet available
Good results in children aged 3-8 years with
criteria :
 Walking but have significant spasticity
 Born prematurely
 Have good intellectual function & good
voluntary control
Relative contraindication : fixed contractures 
may need surgical correction
Physical Therapy
  or prevent problems arising from abnormal muscle tone,
imbalance between opposing muscle groups & abnormal body
balance mechanisms
 A range of regular movement exercises will prevent or
(perhaps more realistically)  degree of muscle / joint
contracture
 Most helpful in early childhood up to age 7 or 8 years
 Postoperative physiotherapy is essential  maximize effects
of surgery & overcome immediate pain, stiffness & weakness
 Positioning & splinting
Disadvantageous positions  hip adduction
 Splints
To prevent muscle contracture, maintain joint position,
improve movement & function, maintaining position
following surgery
Badly fitting splint  does nothing – provokes pain &
spasm &  deformity
 Manipulation & serial casting
Limited role in improving muscle / joint contractures
 relaps is frequent
Operative Treatment
Indications :
 Spastic deformity which cannot be controlled by
conservative measures
 Fixed deformity that interferes with function
 Secondary complications  bony deformities,
dislocation of hip & joint instability
Weak muscles can be augmented by tendon
transfers  gravity plays important part in guiding
choice of tendon transfers
Timing is often crucial
CNS & gait pattern matures around age 7-8 years
Our preferred approach is to avoid ‘little and
often’ surgery in favour of ‘all or none’ philosophy,
but some patients require former & some the latter
Earlier operation may be called for if hip threatens
to dislocate
Regional Survey : Upper Limb
Most typically in child with spastic
hemiplegia or total body involvement
 flexion of elbow, pronation of
forearm, flexion of wrist, clenched
fingers, adduction of thumb
Aimed at improving resting position
of limb & restorating grasp
Elbow Flexion Deformity
If elbow can extend to a right angle
 no treatment
Occasionally necessary to treat a
more marked flexion contracture by
fractional lengthening of biceps &
brachialis tendons  release of
brachialis origin
Forearm Pronation Deformity
Fairly common  subluxation /
dislocation of radial head
Simple release of pronator teres or
tendon can be rerouted round
back of forearm  act as a
supinator
Wrist Flexion Deformity
 Usually in an ulnar direction  improved by
lengthening or releasing FCU
 If extension is weak, released flexor tendon is
transferred into one of wrist extensors
 Severe cases  wrist arthrodesis with excision of
proximal carpal row  cosmetic rather than
functional benefit
Flexion Deformity of The Fingers
 Spasticity of long flexor muscles  clawing
 Flexor tendons can be lengthened individually
 Severe deformity  forearm muscle slide more
appropriate
 If fingers can be unclenched only by
simultaneously flexing wrist, obviously important
not to extend wrist by tendon transfer or fusion
Thumb-in-palm deformity
 Due to spasticity of thumb adductors or flexors (or
both), but later there is also contracture of FPL
 Mild cases  function can be improved by splinting
thumb away from palm, or by operative release of
adductor pollicis & 1st dorsal interosseus muscles
 Resistant deformity  combined lengthening of FPL
& release of thenar muscles, followed by tendon
transfers  reinforce abduction & extension
Neuromuscular disorder
Regional Survey : Lower Limb
SPASTIC HEMIPLEGIA
Foot/ankle
Tibialis anterior  invariably weak 
equinovarus foot deformity
Active plantar flexion to assist knee extension
during stance phase  care when considering
lengthening of gastroknemius / soleus complex
Perform muscle recession rather than tendon
lengthening
Dynamic varus deformity
Treated by a split tibialis anterior
tendon transfer to outer side of foot
 only ½ is transferred  to avoid
risk of overcorrection into valgus
Older children with fixed deformity
 formal muscle lengthening with or
w/o calcaneal osteotomy
Pes Valgus
 May require subtalar arthrodesis
Hip/knee
 Surgery is not usually required
LLD
 Discrepancies in growth  often short irrespective of any joint contracture
 Epiphyseodesis of contralateral distal femoral and/or proximal tibial physes  can improve some
aspects of gait pattern
SPASTIC DIPLEGIA
 Treatment is concentrated on lower limbs
 Very young child  physiotherapy & splintage 
prevent fixed contractures
 Surgery  to correct structural defects (fixed
contracture, hip subluxation), improve gait
 3-4 yo  sitting & walking pattern  interrelationship
between various postural defects, esp lumbar
lordosis/hip flexion, knee flexion/ankle equinus
 Most children will walk but delayed in learning to
master this
 Not walking by 6-7 yo is unlikely to do so
 Non-ambulant children often have orthopedic problems
similar to those with total body involvement
 Walking diplegics  observational gait analysis is
important & computerized gait analysis may have role in
guiding treatment  each limb assessed independently
Hip adduction deformity
 Walks with thighs together, sometimes scissors gait
 May be combined with spastic internal rotation
 Adductor release is indicated if passive abduction <20
degrees on each side
 Medial hamstring lengthening  done first  this alone may
restore some hip abduction
 Most patients open tenotomy of adductor longus & division of
gracilis will suffice  if fails, other adductors be released
 Anterior branch obturator neurectomy shouldn’t be
performed
Hip flexion deformity
 Often associated with fixed knee flexion  walks with
‘sitting’ posture or hyperextension lumbar spine
 Hip deformity >30o  operative
 Walking child  not to weaken hip flexion too much 
intramuscular lengthening of psoas tendon at pelvic brim is
advocated
 Non-walking child  psoas release at level of lesser
trochanter is allowed
 Associated fixed flexion deformity of knee may require
medial hamstring lengthening
Hip internal rotation deformity
 Usually associated with flexion & adduction 
adductor release & psoas lengthening will be
helpful
 After a few years rotation still excessive 
derotation osteotomy of femur (subtrochanteric
or supracondylar)  this may have to be followed
by compensatory rotation osteotomy of tibia
Hip subluxation
 In 30% CP children
 Persistent flexion-adduction deformity  femoral neck
anteversion
 Weak abductors & not fully weightbearing  risk of
acetabular dysplasia & subluxation of joint  in non-
walkers, may be complete dislocation
 Correction of flexion & adduction deformities before 6 yo
may have a role in preventing subluxation
Neuromuscular disorder
Neuromuscular disorder
Older children may need varus-derotation
osteotomy of femur, perhaps combined with
acetabular reconstruction
Longstanding dislocation in non-walker may be
impossible to reconstruct; if discomfort makes
operation imperative, proximal end of femur can be
excised
Adult walking displegic patient  total hip
replacement in cases where painful degenerative
change is affecting function
Knee flexion deformity
 One of commonest deformities
 Usually due to functional hamstring tightness, often aggravated by
hip flexion or weakness of ankle plantar flexion
 Spastic flexion deformity may be revealed only when hip flexed to
90o  hamstrings tightened
 Capsular contracture of knee joint is uncommon
 Gait analysis  deciding hamstrings truly short or only functionally
short
 Fractional lengthening of hamstrings (medial more often)
 improve gait mechanics  risks weakening hip
extension & exacerbating hip flexion/lumbar lordosis 
hamstrings normally assist with hip extension
 Fractional lengthening of semimembranosus can be
combined with detachment & transfer of semitendinosus
to adductor tubercle at distal end of femur
 Good results (Ma et al, 2006) in children with bilateral
spastic flexion deformities >15 o combined with flexed-
knee posture when standing or walking & ability to stand &
walk only with support
Severe flexion deformities (>25-30o)  extension
osteotomy of distal femur or physeal plating
anteriorly
Knee extension is aided by plantarflexionof foot in
walking  important not to weaken triceps surae
by overzealous lengthening of Achilles tendon
Spastic knee extension
Simple tenotomyof proximal end of rectus femoris
External tibial torsion
Supramalleolar osteotomy
First ensure that deformity is not
actually advantageous in
compensating for ankle/hindfoot
deformity
Equinus of the foot
 Usually toe-walks  triggers excessive plantar-flexion-knee
extension couple  manifested as knee hyperextension
 Children with limited dorsiflexion  gastrocnemius is often
more affected than soleus
 Selective fractional lengthening of fascia/muscle is gaining
favour but judicious percutaneous lengthening of Achilles
tendon still popular
 Relative overlengthening  problem, particularly when
associated knee flexion contractures exist
 If varus deformity is present, treatment is as for hemiplegic
patient
 More common deformity  equinovalgus and a ‘rocker-
bottom’ foot  makes use of splints difficult & disrupts
plantarflexion-knee extension couple, exacerbating knee
flexion posture
 Important to note whether hindfoot deformity is reducible or
not
 Calcaneal lengthening or displacement osteotomy but often
subtalar fusion is required
Such surgery must combined with release of tight
structures (eg. Achilles tendon) & possibly peroneal
lengthening & plication of medial structures when
appropriate
External tibial torsion  supramalleolar osteotomy
but remember that externally rotated gait pattern
may be compensating for an inability of foot to
clear the ground when walking because of weak
muscles / stiff joints
Single event multi-level surgery
(SEMLS)
 Usually has problems at all levels
 Enhance mechanical efficiency of gait by combaining
changes at hip, knee & ankle
 Soft tissue & bony surgery to both limbs can be
performed at one sitting or staged over a few weeks
 Postoperative rehabilitation is complex & time-
consuming but results can be very rewarding
Neuromuscular disorder
Total Body Involvement
Hip
 Hip subluxation progressing to dislocation is common
 Adduction & flexion contractures more frequent & severe 
risk of developing subluxation with acetabular dysplasia
 Often ‘windswept’  one hip lying adducted, flexed,
internally rotated while other lies in abduction & external
rotation & often more extended  release hip abductors &
extensors  gluteus maximus & iliotibial band
 Hip subluxation (>30% uncovering of femoral head) may
require femoral varus derotation (& shortening)
osteotomy as well as acetabular procedure for
correction in addition to soft-tissue releases
 Hip has dislocated  open reduction, release soft tissue
& bony realignment
 Alternative  proximal femoral resection
 Complex surgery & high complication rates
Spine / Pelvis
 Scoliosis is very common (>50%)
 Often a long C-shaped thoracolumbar curve  frequently
incorporates pelvis which is tilted obliquely so that one hip is
abducted & other adducted & threatening to dislocate
 Trunk muscle involvement due to CP  major determinant of
developing deformity
 Various forms of non-operative treatment  some cases opt for long-
term use of adapted wheelchair
Indications for surgery :
 Progressive curve >40o in a child >10 yo
 Inability to sit w/o support
 Range of hip movement that will allow child to sit after spinal
stabilization
 Fixation with pedicle screws & rods extending from thoracic spine
to pelvis
 Recreate lumbar lordosis  at least temporarily, exacerbate
hamstring tightness making sitting more difficult
 Complications :
 Neurological defects
 Problems with wound healing
 Implant failure
 This type of spinal surgery   life expectancy, but
demonstrating concurrent improvement in quality of life has
been more difficult to prove
Other joints
 Surgery may be required & follows principles outlined for
hemiplegic & diplegic patient
Adult Acquired Spastic Paresis
Cerebral damage following stroke or head injury 
persistent spastic paresis in adult  can be
accompanied by disturbance of propioception &
stereognosis
Early recuperative stage  physiotherapy &
splintage  prevent fixed deformities  all
affected joints should be put through full range of
movement every day
Botulinum toxin may be beneficial in resistant cases
 Deformities that passively correctible should be splinted
in neutral position until controlled muscle power returns
 Propioception & coordination  occupational th/
 Once max motor recovery has been achieved (9 mo
after stroke but >1 year after brain injury), residual
deformities or joint instability should be considered for
operative
 Sufficient cognitive ability, awareness of body position
in space, good phychological impetus  if lasting result
is to be expected
Lower limbs  principal deformities requiring
correction  equinus or equinovarus of foot,
flexion of knee, adduction of hip
Upper limb  chances regaining controlled
movement <)  common residual deformities 
adduction & internal rotation of shoulder (often
by shoulder pain), flexion of elbow, wrist, MCP
joints
Friedreich’s Ataxia
 Though rare  most common of hereditary ataxias
 Autosomal recessive condition  detected on genetic testing  a triplet expansion  localized to
chromosome 9
 In USA, about 1 in 90 adults is a carrier
 In childhood (rarely adulthood) & all patients develop progressive ataxia of limbs & of their gait
with associated extensor plantar responses but absent knee & ankle reflexes & sensory disturbances
(loss of vibration sense & 2-point discrimination)
 Dysarthria appears within 5 years of onset
Neurological degeneration in spinocerebellar
tracts, corticospinal tracts, posterior columns of
spinal cord & parts of cerebellum
Slowed motor velocities in median & tibial
nerves with absent sensory action potentials in
sural & digital nerves
Painful muscle spasms occur in some patients 
tend to worsen with time
 More common  progressive cavo-varus foot deformity
 rigid, clawed toes & scoliosis
 The earlier onset of disease the greater is risk of
significant curve progression
 In more severe cases, functional & neurological
deterioration may be rapid with cardiomyopathy &
death in early to mid adulthood
 In other more mild cases, surgical correction of foot &
spine deformities may be worthwhile
Lesions of The Spinal Cord
3 major pathways in spinal cord :
 corticospinal tracts (in the anterior
columns)  motor neurons
 spinothalamic tracts  sensory neurons
 pain, touch, temperature
 posterior column tracts  deep
sensibility  joint position, vibration
Neuromuscular disorder
Clinical features
 True lesions  UMN spastic paresis & often a fairly precise
sensory level
 Extradural compressive lesions will often involve nerve
roots  combination of UMN & LMN signs
 Weakness & numbness with loss of balance & possibly
alteration in bowel or bladder control, impotence
 Several 'classical' patterns are recognized
Cervical cord compression
UMN symptoms in lower limbs (stiffness &
change in gait pattern) & LMN signs in upper
limbs (numbness & clumsiness)
Pain  variable feature
Bladder symptoms are of frequency &
incontinence more commonly than retention
NEUROMUSCULAR
DISORDER
A central cord syndrome
Hyperextension injury in middle-aged patient
with longstanding cervical spondylosis, or may
develop in syringomyelia
Disproportionately more UMN weakness in upper
limbs compared to lower limbs  bladder
dysfunction & a variable sensory loss below
lesion
 Thoracic cord compression
UMN paralysis affecting lower limbs  variable sensory
loss depending on degree of involvement of dorsal
columns or spinothalamic tracts
 Lumbar cord compression
Spinal cord terminates around L1  conus medullaris or
cauda equina or both  mixture of UMN & LMN signs
 Typical cauda equina syndrome  lower limb
weakness, absent reflexes, impaired sensation & urinary
retention
Brown-Sequard lesion
 Pure form  very unusual  incomplete hemispherical
cord lesion :
 Below lesion  ipsilateral UMN weakness & posterior
column dysfunction, contralateral loss of skin
sensibility
 At level of lesion  ipsilateral loss of sensibility
 Less pure forms  common
Neuromuscular disorder
NEUROMUSCULAR
DISORDER
Spinal shock
Acute cord lesions at any level  flaccid paralysis which resolves over time, usually to reveal more
typical UMN signs associated with cord injury
Causes of spinal cord dysfunction
Neuromuscular disorder
Diagnosis & management
Traumatic & compressive lesions  most likely
to be seen
Plain x-rays  structural abnormalities of spine;
cord compressioncan  myelography alone /
combined with CT
Intrinsic lesions of cord  blood tests, CSF
examination & MRI
Acute compressive lesions  urgent diagnosis &
treatment if permanent damage is to be
prevented
Bladder dysfunction is ominous  motor &
sensory signs may improve after decompression
Loss of bladder control >24 hours  irreversible
Any spinal injury may be associated with
cord damage  great care in transporting &
examining patient
In early period of 'spinal shock'  flaccid
paralysis, with or without priapism
Plain x-rays seldom show full extent of bone
displacement  much better by CT / MRI
Unstable injuries  operative decompression
and/or stabilization; stable injuries 
conservatively
Many centres consider use of corticosteroids
 reducing degree of permanent
neurological damage  side effects : GI
haemorrhage & arrascular necrosis
 Epidural abscess  surgical
emergency  acute pain & muscle
spasm, fever, leucocytosis,  ESR
 X-rays  disc space narrowing &
bone erosion  immediate
decompression & antibiotics
 Acute disc prolapse  unilateral
symptoms & signs
 Complete lumbar disc prolapse 
cauda equina syndrome  urinary
retention & overflow
 Spinal canal obstruction  MRI
 Operative discectomy  urgent
 Chronic discogenic disease 
narrowing of intervertebral
foramina & compression of nerve
roots (radiculopathy), bone
hypertrophy, pressure on spinal
cord (myelopathy)  x-ray & MRI
 operative decompression
 Spinal stenosis  direct pressure
on cord / nerve roots, vascular
obstruction, ischaemic neuropathy
during hyperextension of lumbar
spine  'tiredness', weakness,
aching / paraesthesia in lower
limbs after standing / walking for
a few minutes  relieved by
bending forward, sitting /
crouching so as to flex lumbar
spine
 Congenital narrowing of
spinal canal  rare, except
in developmental disorders
(achondroplasia)  bony
decompression of nerve
structures
 Vertebal disease  TB /
metastatic disease  cord
compression & paraparesis
 x-ray  needle biopsy for
confirmation  anterior
decompression & internal
stabilization, radiotherapy,
corticosteroids, narcotics
 Spinal cord tumours 
comparatively rare 
progressive paraparesis
 X-rays  bony erosion, widening
of spinal canal / flattening of
vertebral pedicles
 Widening of intervertebral
foramina  typical of
neurofibromatosis  operative
removal of the tumour
 Intrinsic lesions of cord  slowly
progressive neurological signs 
tabes dorsalis & syringomyelia 
neuropathic joint destruction
 Tabes dorsalis  late
manifestation of syphilis 
degeneration of posterior
columns of spinal cord 
'lightning pains' in lower limbs
 Sensory ataxia  stamping
gait; loss of position sense &
pain sensibility; trophic
lesions in lower limbs;
progressive joint instability;
almost painless destruction of
joints (Charcot joints)
 No treatment for cord
disorder
 Syringomyelia  long cavity
(the syrinx) filled with CSF
develops within spinal cord
 usually the cause is
unknown, sometimes
associated with tumours / SCI
& congenital anomalies
(hidrocephalus & herniation
of cerebellar tonsils)
 Symptoms & signs  most
noticeable in upper limbs 
expanding cyst presses on
anterior horn cells 
weakness & wasting of hand
muscles
 Destruction of decussating
spinothalamic fibres in centre of
cord  sensory loss in upper limbs:
impaired response to pain &
temperature but preservation of
touch
 Trophic lesions in fingers &
neuropathic arthropathy ('Charcot
joints') in upper limbs
 CT  expanded cord & syrinx 
MRI
 Deterioration may be slowed down
by decompression of foramen
magnum
SPINA BIFIDA
 Congenital disorder  2
halves of posterior vertebral
arch fail to fuse at ≥1 levels
 Neural tube defect, or spiral
dysraphism  1st month of
foetal life  lumbar /
lumbosacral
 Most severe form  major
neurological problems in
lower limbs & incontinence
SPINA BIFIDA
Spina bifida occulta
 Mildest forms of dysraphism 
midline defect between the
laminae & nothing more
 Usually L5
 Telltale defects in overlying skin
 dimple, pit, tuft of hair
 Tethering of conus medullaris
below L1, splitting of spinal cord
(diastematomyelia), cysts /
lipomas of cauda equina
Spina bifida cystica  Vertebral laminae  missing &
contents of vertebral canal
prolapse through defect
 Abnormality takes 1 of several
forms
 Least disabling  meningocele
 5%
 Duramater open posteriorly,
meninges intact, CSF-filled
meningeal sac protrudes under
skin
 Spinal cord & nerve roots
remain inside vertebral canal
 no neurological abnormality
Myelomeningocele  Most common & serious abnormality
 lower thoracic spine /
lumbosacral
 Part of spinal cord & nerve roots
prolapse into meningeal sac
 Neural tube fully formed & sac
covered by membrane and/or skin
 closed myelomeningocele
 In others cord in unfolded neural
plate forming roof of sac  open
myelomeningocele  neurological
deficit distal to level of lesion
 If neural tissue exposed to air 
infected  more severe
abnormality & death
Hydrocephalus
Distal tethering of cord  herniation of
cerebellum & brain-stem through foramen
magnum  obstruction to CSF circulation &
hydrocephalus
Ventricles dilate & skull enlarges by separation
of cranial sutures
Persistently raised intracranial pressure 
cerebral atrophy & learning difficulties
lncidence & screening
Isolated laminar defects  >5% of lumbar
spine x-rays
Cystic spina bifida  rare  2-3/1000 live
births
If 1 child is affected  risk for future
siblings significantly 
Neuromuscular disorder
Neural tube defects  high levels of AFP in
amniotic fluid & serum  ANC 15-18th week of
pregnancy.
Maternal blood testing  15-18 weeks &
followed by an amniocentesis if necessary
Mid-term high resolution USG  95%
Folic acid 400 micrograms/day  continuing
through the first 12 weeks of pregnancy
Neuromuscular disorder
Neuromuscular disorder
ClinicaI features
EARLY DIAGNOSIS
 Spina bifida occulta  enuresis, urinary frequency /
intermittent incontinence; weakness, some loss of
sensibility in lower limbs
 Plain x-rays  laminar defect & any associated
vertebral anomalies; midline ridge of bone 
bifurcation of cord (diastematomyelia)
 Intraspinal anomalies  MRI
Spina bifida cystica
 Saccular lesion overlying lumbar spine
 Open myelomeningoceles  plum coloured skin
 1/3 infants  complete LMN paralysis & loss of sensation &
sphincter control below affected level
 X-rays & CT  extent of bony lesion + other vertebral
anomalies
 MRI  define neurological defects
Clinical features in older children
Clawing toes, change in gait pattern,
incontinence / abnormal sensation  tethered
cord syndrome
MRI with gadolinium
Neurosurgical release
Liable to suffer fractures after minor injuries
Treatment
 Intrauterine surgery  closure of defect
 Formal neurological closure  <48 hours of birth 
prevent drying, ulceration, infection
 Some centres avoid urgent operation if above L1 / very
severe spinal deformities / marked hydrocephalus
 90% need VP shunt   risk of further damage to CNS
Neurological status changes unexpectedly 
shunt infection / blockage
Physiotherapy and/or splinting  mainstays of
early treatment
Vast majority of patients  urological problems
 catheters / urinary diversion  botulinum
toxin injections   capacity & improve
continence
Neuromuscular disorder
ORTHOPAEDIC MANAGEMENT
 Except in mildest cases, late functional outcome
cannot be predicted until child is assessed
intellectually & neuromuscular unction  3-4
years
 For many patients, ability to sit comfortably is
more important than to stand awkwardly
 Best predictor of walking ability & function 
motor level of paralysis
Lesions <L4  quadriceps control & active knee
extension
Higher lesions  wheelchair
Immobilization & muscle imbalance  joint
deformity & risk ofpathological #
Latex allergy is present in some children with spina
bifida
Th/: antihistamines and/or corticosteroids
Spine
 Scoliosis and/or kyphosis is common in children
with myelomeningocele  muscle weakness &
imbalance, congenital vertebral anomalies (20 %
cases) & tethered cord syndrome
 Distal tethering of cord / other neural elements
 almost inevitable after repair of
myelomeningocele  harmless  pain &
progression of neurological dysfunction  PPS
Kyphosis
 Stretching & breakdown / chronic ulceration of
overlying skin posteriorly & compression of
abdominal & thoracic viscera anteriorly
 Treatment  difficult  localized vertebral
resection & arthrodesis
 Cord at affected level ofter non-functioning 
risks of further neurological insult influencirrg
outcome are small
Paralytic scoliosis
 Long C-shaped curve  progressive  sitting
difficult
 Unlikely to respond to brace
 Molded seat inserts for wheelchair  help  rate
of curve deterioration
 Surgery via anterior, posterior or combined
apprroach; fusion to pelvis
Hip
 General aim  to secure hips  enable child
to stand in calipers & to sit comfortably
 Neurological lesion >L1  all muscle groups
are flaccid  splintage & wheelchair
 Lesions <S1  hip flexion contracture 
elongation of psoas tendon + detachment of
flexors from ilium  Soutter operation
Neuromuscular disorder
Neuromuscular disorder
Muscle imbalance  bilateral hip
dislocation
‘In between’ lesions  muscle imbalance 
hip sublux / dislocate
Retaining hip movement may be more useful
than striving for hip reduction by multiple
operations
Knee
Aim  straight knee for callipers & using gait-
training devices
Older children  prolonged sitting  fixed
flexion  stretching (by distraction) fails  ≥1
hamstrings lengthened, divided / reinserted into
femur / patella; posterior capsular release
Hyperextension contracture & hamstring
tendons subluxed anteriorly  physiotherapy &
serial casting  V-Y quadricepsplasty &
hamstring lengthening
Walking patients  valgus knee, torsional
abnormalities in lower limb
Secondary joint instability  further exacerbate
problems of walking  forearm crutches &
swing-through gait
Foot
Most common problem
Aim  mobile foot, healthy skin & soft tissues 
can be held / braced in plantigrade position
Flail foot / balanced paralysis / weakness 
accurately made orthoses (eg. ankle-foot orthosis),
well-fitting ankle boots
Equinovarus deformity  standard treatment :
aggressive soft-tissue release
Bony procedures  for residual / recurrent
deformity in older child
Vertical talus deformity  ‘reverse Ponseti’
& transfer of tibialis anterior tendon to neck
talus
Toe deformities  ‘orthopaedic shoes’ with
high toe box
POLIOMYELITIS
 Acute infectious viral disease
 Spread by oropharyngeal route
 10% exhibit any symptoms at all & 1% effects on anterior
horn cells of spinal cord & brain stem  LMN paralysis
 Acute illness resembling meningitis to paralysis, slow
recovery / convalescence, long period of residual
paralysis
 At any age but most commonly in children
Acute illness  fever, headache
 1/3 patients  sore throat, mild headache, slight pyrexia 5-7
days before  neck stiffness
 Patient curled up  passive stretching provoke painful spasms
Paralysis  peak at 2-3 days  breathing & swallowing difficulty
 Pain & pyrexia subside after 7-10 days
 Infective for 4 weeks from onset
Recovery & convalescence  first 6 months
up to 2 years
Residual paralysis  some degree of
asymmetric flaccid (LMN) paralysis /
unbalanced muscle weakness  joint
deformities & growth defects
Sensation intact, but limb often cold & blue
Post-polio syndrome  up to 50% reactivation of
virus  progressive muscle weakness in old &
new muscle groups  unaccustomed fatigue
Confirmed poliomyelitis + neurological stability
min 15 years
The older the child at onset, more severe the
disease & more likely the adult would develop
PPS
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
Early treatment
Acute phase  isolated, symptomatic treatment
for pain & muscle spasm, gentle passive stretching,
artificial respiration
Acute illness settles  physiotherapy
Between exercise periods, splintage
Muscle charting at regular intervals until no further
recovery detected
Late treatment
 Isolated muscle weakness without deformity 
instability (quadriceps paralysis) / loss of complex
function (thumb opposition  tendon transfer)
 Passively correctible deformity  appropriate tendon
transfer may solve problem permanently
Muscle usually loses 1 grade power when transferred
Grade 3 muscle  tenodesis  reduce deformity by
gravity
Neuromuscular disorder
Fixed deformity  operatively stabilize joint
 arthrodesis  ankle, foot, paralytic scoliosis
Occasionally beneficial  equinus foot to
compensate mechanically for quadriceps
weakness
Flail joint  if unstable, permanent splintage /
arthrodesis
Neuromuscular disorder
Shortening
 Normal bone growth depends on normal muscle
activity  children with poliomyelitis in early
years can develop difference in leg length
 LLD 3-5 cm  shoe raise
 LLD can be mitigated by well-timed
epiphyseodesis in normal limb
Disturbance of skeletal modelling 
torsional / angular deformities in sagittal /
coronal plane
Muscle & joint contractures may aggravate
effects of bone distortion
Vascular dysfunction  large chilblains 
sympathectomy
Shoulder
Strong scapular muscles  abduction at
shoulder can be restored by arthrodesing
gleno-humeral joint (50o abducted & 25o
flexed)
Contracted adductors may need division
Elbow & forearm
 Normal power in anterior forearm muscles  common
flexor origin moved more proximally on distal humerus
 Strong pectoralis major  lower half of muscle detached
& join biceps tendon
 Pronation of forearm  transposing active FCU tendon
across front of forearm to radial border
 Supination loss  transposing FCU across back of forearm
to distal radius
Wrist & hand
 Deformity / instability  arthrodesis
 Any active muscles can be used to restore
finger movement
 Opposition weakness  flexor superficialis
transfer  tendon wound round FCU,
threaded across palm & fixed to distal end of
1st MC
Lister GD: The Hand: Diagnosis and Indications, ed 2. Edinburgh, Scotland: Churchill
Trunk
Unbalanced paralysis  scoliosis  long
thoracolumbar curve  lumbosacral junction
 pelvic obliquity
Most effective operation : anterior &
posterior instrumentation & fusion
Hip
 Paralysis usually <5 yo  persistent anteversion of femoral
neck, coxa valga, underdevelopment of acetabular socket
 Keys : reduce any scoliotic pelvic obliquity, overcome muscle
imbalance, correct prox femoral deformity by
intertrochanteric / subtrochanteric osteotomy,
acetabuloplasty to deepen acetabular socket
 Fixed abduction with pelvic obliquity  fascia lata & ITB may
need division
Knee
Instability  relative weakness of knee
extensors
Good extensor power hip + good foot
plantarflexion power (or fixed equinus) + knee
thrust into hyperextension  unaided walking
Weak hip / ankle joints  full-length calliper /
supracondylar extension osteotomy of femur
Fixed flexion with flexors stronger than extensors is
more common
Flexor-to-extensor transfer  hamstring muscles to
patella / quadriceps tendon
If flexors not strong enough  supracondylar
extension osteotomy
Genu recurvatum  supracondylar extension
osteotomy / excise patella & slot into upper tibia
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
Foot
 Foot drop  ankle-foot orthosis / below-knee calliper
 Varus, valgus, calcaneocavus  fusion + tendon re-
routing
 Varus, valgus  Grice, Dunn
 Foot drop  Lambrinudi
 Calcaneocavus  Elmslie
Grice
Neuromuscular disorder
Neuromuscular disorder
Neuromuscular disorder
Dunn
A, Position of skin incision (broken
line) and amount of bone resected
(colored area). B, Position of bones
after surgery. Foot (except for
talus) has been displaced
posteriorly at subtalar joint so that
Lambrinudi
Neuromuscular disorder
Elmslie
Neuromuscular disorder
Neuromuscular disorder
Claw toes
If mobile  transfer toe flexors to extensors
If fixed  IP joint should be arthrodesed in
straight position & long extensor tendons
reinserted into metatarsal necks
Motor Neuron Disorders
Motor neuron disease (amyotrophic
lateral sclerosis)
 Degenerative disease, unknown etiology
 UMN & LMN symptoms & signs
 Middle age patient
 Dysarthria, difficulty in swallowing, muscle weakness,
wasting, exaggerated reflexes
 Muscle cramps, atrophy, fasciculation
Progressive, incurable
Some have frontotemporal
dementia / pseudobulbar
effect  emotional
lability
Most die within 5 years 
respiratory weakness +
aspiration pneumonia
Spinal Muscular Atrophy
 Heritable (defect on long arm of chromosome 5)
 Widespread degeneration of anterior horn cells in cord 
progressive LMN weakness
 Commonest : Werdnig-Hoffman disease  autosomal
recessive  floppy & weak baby, difficult feeding &
breathing  death in 1 year
 Less severe : Kugelberg-Welander disease  dominant /
recessive  adolescents / young adult
Werdnig-Hoffman disease
Kugelberg-Welander disease
Peripheral Neuropathies
Classification
 By anatomical level & distribution
 >40% cases  no specific cause is found
 Radiculopathy  nerve roots  trauma,
intervertebral disc herniation / bony spur, SOL of
spinal canal, root infection
 Plexopathy  direct trauma, compression by local
tumours, entrapment in thoracic outlet syndrome,
viral infection
Pancoast tumour
Entrapment in thoracic outlet syndrome
Neuralgic amyotrophy
A. On the left: atrophy of supraspinatus and infraspinatus muscles and rhomboid
muscles (white arrow); on the right: scapular tilting and rotation caused by serratus
anterior muscle weakness (white arrow with *)
B. On the right: severe scapular winging caused by serratus anterior paralysis
C. On the left: atrophy of supraspinatus and infraspinatus muscles
(white arrow), and trapezius muscle (white arrow with *) showing
underlying rhomboid muscles
D. Severe atrophy of the deltoid muscle (white arrow) and moderate
atrophy of the biceps brachii muscle (white arrow with *)
Distal neuronopathy :
Mononeuropathy  single nerve, mixed
sensorimotor
Multiple mononeuropathy  several isolated
nerves  leprosy, diabetes, vasculitis
Polyneuropathy  widespread symmetrical
dysfunction
Pathology
 Large nerve fibres   >4 µm  myelinated
 Small fibres   <4 µm  unmyelinated
 Acute interruption of axonal continuity
 Axonal degeneration
 Demyelination
Acute Axonal Interruption
After nerve division
Loss of motor & sensory  immediate & complete
Muscle fibres will degenerate if nerve conduction
not restored in 2 years
NCV & EMG
Axon grows ±1 mm / day, often incomplete
Chronic Axonal Degeneration
 Non-traumatic
 Large fibres  ‘stocking’ & ‘glove’ numbness, altered postural reflexes,
ataxia, muscle weakness & wasting
 Feet & legs before hands & arms
 NVC  CMAP & SNAP  & EMG
 Small fibres  orthostatic hypotension, cardiac arrhythmias,  peripheral
limb perfusion, ischaemia, limb infection, burning dysaesthesias
Demyelinating Neuropathies
 Nerve entrapment
syndrome & blunt soft-
tissue trauma
 Sensory and/or motor
dysfunction distal to
lesion
 Recovery <6 weeks
 Rare, exeption: Guillain-
Barre syndrome
Clinical features
‘pins & needles’ numbness, limb ‘going to
sleep’, ‘burning’, shooting pains, restless legs
Weakness, clumsiness, loss of balance in walking
Pain sensibility & propioception depressed 
Charcot joints
Peripheral neuritis
Hereditary neuropathies
Hereditary sensory neuropathy
Dominant or recessive trait
Neuropathic joint disease & ulceration of
feet
Cycle of painless injury & progressive
deformity  severe disability
Neuromuscular disorder
Hereditary motor & sensory neuropathy (HMSN)
Peroneal muscular atrophy, Charcot-Maria-Tooth
disease, benign forms of spinal muscular atrophy
Autosomal dominant
HMSN type I  children  difficulty walking, claw
toes, pes cavus / cavovarus  confirmed by
demyelination on sural nerve biopsy / genetic test
of blood samples
"Stork legs"  most noticeable distal to knees
Neuromuscular disorder
 HMSN type II  adolescents & young adults  much less disabling than
type I  affect only lower limbs  mild pes cavus & wasting of peronei
 Early stages  foot & ankle orthoses
 Claw toes  transfer toe flexors to extensors, with / wo fusion of IP joints
 Clawing of big toe  Robert Jones procedure  transfer EHL to metatarsal
neck & fusion of IP joint
 Cavus deformity  calcaneal / dorsal mid-tarsal osteotomy / triple
arthrodesis
Robert Jones procedure
Familial liability to pressure
palsy (HNPP)
Dominant disorder
Multiple mixed entrapment
mononeuropathies  CTS + ulnar nerve palsy
Friedreich’s Ataxia
 Autosomal recessive
 Spinocerebellar ataxia: spinocerebellar dysfunction and may be also be
degeneration of the posterior root ganglia and peripheral nerve.
 Present at 6 y.o, with ataxic gait, lower limb weakness.
 The muscle weakness is progressive -> at 20 y.o. use wheelchair -> die due to
cardiomyopathy before 45 y.o
NEUROMUSCULAR
DISORDER
Metabolic Neuropathies
Diabetic Neuropathy
 Asscociated with hyperglycemia,
interfere axon and schwann cell
function -> demyelination and axonal
degeneration
 The onset is insidious
 Condition often goes undiagnosed until
patients start complaining of numbness
and paraesthesiae in the feet and
lower legs
 Complications: neuropathic ulcers of
the feet, regional osteoporosis,
insufficiency fractures of the foot
bones, or Charcot joints in the ankles
and feet
Treatment:
 skin care, management of fractures and splintage or
arthrodesis of grossly unstable or deformed joints
Alcoholic neuropathy
 the main cause is the accompanying nutritional
deficiency, especially thiamine deficiency
 Symptoms: ‘burning’, paraesthesiae, numbness
and muscle weakness in the feet and legs
 Treatment: nutritional supplementation,
administration of thiamine, protection from
trauma
INFECTIVE NEUROPATHY
 caused by the varicella
 dormant for many years in the dorsal root ganglia, is then
reactivated and migrates down the nerve
 Symptoms:
 the patient develops severe unilateral pain in the distribution of
several adjacent nerve roots
 Days or weeks later an irritating vesicular rash appears;
characteristically it trails out along the dermatomes corresponding
to affected nerves
Herpes Zoster (shingles)
This patient was treated for
several weeks for ‘sciatica’
– then the typical rash of
shingles appeared
Neuralgic amyotrophy (acute brachial
neuritis)
 unusual cause of severe shoulder girdle pain and
weakness is believed to be due to a para-
infectious disorder of one or more of the cervical
nerve roots and the brachial plexus
 Pain in the shoulder and arm is typically sudden in
onset,intense and unabating
 Other symptoms are paraesthesiae in the arm or
hand and weakness of the muscles of the
shoulder, forearm and hand
 Winging of the scapula (due to serratus
anterior weakness), wasting of the shoulder
girdle muscles, and occasionally involvement
of more distal arm muscles may be profound,
becoming evident as the pain improves
 Sensory loss and paraesthesiae in one or more
of the cervical dermatomes is common
 no specific treatment; pain is controlled with
analgesics
Guillain–Barré syndrome
(acute inflammatory demyelinating polyneuropathy –
AIDP)
 acute demyelinating motor and
sensory (though mainly motor)
polyneuropathy
 can occur at any age and usually
appears two or three weeks after
an upper respiratory or
gastrointestinal infection –
probably as an autoimmune
reaction
 Cerebrospinal fluid analysis may
show a characteristic pattern:
elevated protein concentration in
the presence of a normal cell
count
 Nerve conduction studies may
show conduction slowing or block;
in severe cases there may be EMG
signs of axonal damage
 Treatment consists essentially of
bed rest, pain-relieving
medication and supportive
management to monitor, prevent
and deal with complications such
as respiratory failure and difficulty
with swallowing
Leprosy
 Mycobacterium leprae, causes a
diffuse inflammatory disorder of
the skin, mucous membranes and
peripheral nerves
 In tuberculoid leprosy, anaesthetic
skin patches develop over the
extensor surfaces of the limbs;
loss of motor function leads to
weakness and deformities of the
hands and feet
 Treatment by combined
chemotherapy (mainly rifampicin
and dapsone) is continued for 6
months to 2 years
PAIN
Pain receptors
 stimulated by mechanical
distortion, by chemical, thermal
or electrical irritation, or by
ischaemia
 Musculoskeletal pain associated
with trauma or inflammation is
due to both tissue distortion and
chemical irritation (local release
of kinins, prostaglandins and
serotonin)
 Visceral nociceptors respond to
stretching and anoxia
Pain perception
Pain transmission
 transmitted via both myelinated axons
 From the dorsal horn synapses in the cord,
some fibres participate in ipsilateral reflex
motor and autonomic activities while others
connect with axons in the contralateral
spinothalamic tracts that run to the thalamus
and cortex
Pain modulation
 Pain impulses may be suppressed or inhibited
by
 simultaneous sensory impulses travelling via
adjacent axons
 impulses descending from the brain
 pain impulses are ‘sorted out’ – some of them
blocked, some allowed through – in the dorsal
horn of the cord
 certain morphine-like compounds (endorphins
and enkephalins), normally elaborated in the
brain and spinal cord, can inhibit pain
sensibility
Pain threshold
 No fixed threshold for any individual
 threshold is lowered by fear, anxiety,
depression, lack of self-esteem and mental or
physical fatigue
 elevated by relaxation, diversion, reduction
of anxiety and general psychological support
Acute pain
 Severe acute pain, is accompanied
by an autonomic ‘fight or flight’
reaction:
 Increased pulse rate
 peripheral vasoconstriction
 Sweating
 Rapid breathing
 muscle tension
 Anxiety
 Treatment is directed at:
(1) removing or counteracting the
painful disorder
(2) splinting the painful area
(3) making the patient feel
comfortable and secure
(4) administering analgesics, anti-
inflammatory drugs or – if
necessary – narcotic preparations
(5) alleviating anxiety
Chronic pain
 occurs in degenerative and
arthritic disorders or in malignant
disease and is accompanied by
vegetative features such as
fatigue and depression
 Treatment again involves
alleviation of the underlying
disorder if possible and general
analgesic therapy
 Need rehabilitation and
psychologically support
Complex regional pain syndrome (CRPS)
 pain out of proportion (in both
intensity and duration) to the
precipitating cause,vasomotor
instability, trophic skin changes,
regional osteoporosis and
functional impairment
 Causes are trauma (often trivial),
operation or arthroscopy, a
peripheral nerve lesion,
myocardial infarction, stroke and
hemiplegia
Neuromuscular disorder
PATHOGENESIS
 sympathetic overactivity
 Abnormal cytokine release, neurogenic
inflammation, sympathetic-mediated
enhancement of pain responses
CLINICAL FEATURES
 Complains of burning pain, and sometimes
cold intolerance, in the affected area –
usually the hand or foot, sometimes the knee
or hip, and sometimes the shoulder in
hemiplegia
 Local redness and warmth, sometimes
changing to cyanosis with a blotchy, cold and
sweaty skin
 X-rays are at first usually normal
 Causalgia is a severe form of regional pain,
usually seen after a nerve injury
 Pain is intense, often ‘burning’ or
‘penetrating’ and exacerbated by touching,
jarring or sometimes even by a loud noise
TREATMENT
 Mild cases often respond to a simple regimen
of reassurance, anti-inflammatory drugs and
physiotherapy
 Administration of corticosteroids, calcium
channel blockers and tricyclic antidepressants
 If there is no improvement after a few weeks
sympathetic blockade often helps
 Psychological treatment may help them to
deal with the emotional distress and anxiety
and to develop better coping strategies
Chronic pain syndrome
 well-marked features of
depression, or complaints of
widespread somatic illness (pain in
various parts of the body,
muscular weakness,
paraesthesiae, palpitations and
impotence)
 Treatment is always difficult and
should, ideally, be managed by a
team that includes a specialist in
pain control, a psychotherapist, a
rehabilitation specialist and a
social worker
 Pain may be alleviated by a
variety of measures:
(1) analgesics and anti-inflammatory
drugs;
(2) local injections to painful areas;
(3) local counter-irritants;
(4) acupuncture;
(5) transcutaneous nerve stimulation;
(6) sympathetic block;
(7) surgical interruption of pain
pathways
FIBROMYALGIA
 complain of pain and tenderness in the
muscles and other soft tissues around the
back of the neck and shoulders and across the
lower part of the back and the upper parts of
the buttocks
NEUROMUSCULAR
DISORDER
Muscular Dystrophies
 Duchenne’s muscular dystrophy
 Limb girdle dystrophies
 Facioscapulohumeral dystrophy
Duchenne’s muscular dystrophy
 Inheritance with recessive
transmission
 seen only in boys (or in girls with
sex chromosome disorders)
 Clinical Appereance
 difficulty standing and
climbing stairs, he cannot run
properly and he falls
frequently
 Weakness begins in the
proximal muscles of the
lower limbs and progresses
distally, affecting particularly
the glutei, the quadriceps
and the tibialis anterior,
giving rise to a wide-based
stance and gait with the feet
in equinus, the pelvis tilted
forwards, the back arched in
lordosis and the neck
extended
 Characteristic feature is the
child’s method of rising from
the floor by climbing up his
own legs (Gowers’ sign); this
is due to weakness of the
gluteus maximus and thigh
muscles
 Cardiopulmonary failure is
the usual cause of death,
generally before the age of
30 years
 Confirmation is achieved by
muscle biopsy and genetic
testing with a DNA
polymerase chain reaction.
Treatment
 While the child can still walk -> physiotherapy and
splintage
 Corticosteroids are useful in preserving muscle strength
 If scoliosis is marked (more than 30 degrees),
instrumentation and spinal fusion helps to maintain
pulmonary function and improves quality of life
although not necessarily lifespan
NEUROMUSCULAR
DISORDER
BECKER MUSCULAR DYSTROPHY
 X-linked recessive disease, is similar to but milder
than Duchenne’s dystrophy
 Dystrophin is decreased and/or abnormal in character
 The muscles of facial expression are not affected and
neither are the muscles controlling bowel or bladder
function or swallowing
LIMB GIRDLE DYSTROPHY
 characterized by :
 weakness of the pelvic and
shoulder girdle muscles
 usually start in late adolescence
 causes a waddling gait and
difficulty in rising from a low chair
 pectoral girdle weakness makes it
difficult to raise the arms above
the head
Treatment
 Physiotherapy
 Splintage to prevent contractures,
 operative correction when
necessary
FACIOSCAPULOHUMERAL DYSTROPHY
 autosomal dominant condition
with very variable expression
 muscle weakness is first seen in
 the face (inability to purse the
lips or close the eyes tightly)
 followed by weakness of
scapular muscles causing winging
of the scapula and difficulty with
shoulder abduction
 condition is due to gene deletion
on the long arm of chromosome 4
MYOTONIA
 persistent muscle contraction after cessation of voluntary effort
 Two type:
1. DYSTROPHIA MYOTONICA
2. MYOTONIA CONGENITA
DYSTROPHIA MYOTONICA
 autosomal dominant disorder
with an incidence of about 1
in 7000
 muscle stiffness for some
years
 systemic features appear –
diabetes, cataracts and
cardiorespiratory problems –
and by middle age patients
are often severely disabled
 Treatment is essentially
palliative but foot
deformities may need
manipulation and splintage
MYOTONIA CONGENITA
 inherited by autosomal recessive
transmission
 appear in childhood and usually
progress slowly
 typically this is worse after
periods of inactivity and is
relieved by exercise
 triggered by exposure to cold and
can cause pain (‘muscle cramps’)
 There is no specific treatment for
this condition.
 Patients are advised about
avoiding aggravating activities.
Neuromuscular disorder
Neuromuscular disorder

Weitere ähnliche Inhalte

Was ist angesagt? (20)

Cerebellar disorders
Cerebellar disordersCerebellar disorders
Cerebellar disorders
 
NERVE CONDUCTION STUDIES, ELECTROMYOGRAPHY
NERVE CONDUCTION STUDIES, ELECTROMYOGRAPHYNERVE CONDUCTION STUDIES, ELECTROMYOGRAPHY
NERVE CONDUCTION STUDIES, ELECTROMYOGRAPHY
 
Physiology of movement
Physiology of movementPhysiology of movement
Physiology of movement
 
Approach to the_patient_with_myopathy
Approach to the_patient_with_myopathyApproach to the_patient_with_myopathy
Approach to the_patient_with_myopathy
 
NCV AND EMG
NCV AND EMGNCV AND EMG
NCV AND EMG
 
motor neuron disease
motor neuron diseasemotor neuron disease
motor neuron disease
 
Disorders of the neuromuscular junction
Disorders of the neuromuscular junctionDisorders of the neuromuscular junction
Disorders of the neuromuscular junction
 
H Reflexes in Clinical Practice
H Reflexes in Clinical PracticeH Reflexes in Clinical Practice
H Reflexes in Clinical Practice
 
Polyneuropathy
PolyneuropathyPolyneuropathy
Polyneuropathy
 
Management of motor neuron disease
Management of motor neuron diseaseManagement of motor neuron disease
Management of motor neuron disease
 
Neuroplasticity
NeuroplasticityNeuroplasticity
Neuroplasticity
 
Nerve Conduction Studies- Lower Leg
Nerve Conduction Studies- Lower LegNerve Conduction Studies- Lower Leg
Nerve Conduction Studies- Lower Leg
 
Overview of Nerve Conduction Study
Overview of Nerve Conduction StudyOverview of Nerve Conduction Study
Overview of Nerve Conduction Study
 
Neural control of locomotion
Neural control of locomotionNeural control of locomotion
Neural control of locomotion
 
Peripheral neuropathy
Peripheral neuropathyPeripheral neuropathy
Peripheral neuropathy
 
peripheral nerve lesions
peripheral nerve lesionsperipheral nerve lesions
peripheral nerve lesions
 
History SFEMG
History SFEMGHistory SFEMG
History SFEMG
 
Cerebral palsy and its types
Cerebral palsy and its typesCerebral palsy and its types
Cerebral palsy and its types
 
Fundamentals of nerve conduction study
Fundamentals of nerve conduction studyFundamentals of nerve conduction study
Fundamentals of nerve conduction study
 
Nerve conduction study
Nerve conduction studyNerve conduction study
Nerve conduction study
 

Andere mochten auch

Neuromuscular Disorders affecting the orofacial region
Neuromuscular Disorders affecting the orofacial regionNeuromuscular Disorders affecting the orofacial region
Neuromuscular Disorders affecting the orofacial regionVibhuti Kaul
 
Neuromuscular Disorders
Neuromuscular DisordersNeuromuscular Disorders
Neuromuscular DisordersAnna Maria
 
Neuromuscular Junction Disease
Neuromuscular Junction DiseaseNeuromuscular Junction Disease
Neuromuscular Junction DiseaseMiami Dade
 
Evaluation and investigation of Neuromuscular disorders
Evaluation and investigation of Neuromuscular disordersEvaluation and investigation of Neuromuscular disorders
Evaluation and investigation of Neuromuscular disordersPro Faather
 
Neuromuscular disorders for dental management
Neuromuscular disorders for dental managementNeuromuscular disorders for dental management
Neuromuscular disorders for dental managementPatience Monde
 
Neuromuscular disorders in children (2)
Neuromuscular disorders in children (2)Neuromuscular disorders in children (2)
Neuromuscular disorders in children (2)shivani1305
 
PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...
PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...
PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...shuchij10
 
Neuromuscular Disorders Respiratory Complications and Assessment
Neuromuscular Disorders Respiratory Complications and AssessmentNeuromuscular Disorders Respiratory Complications and Assessment
Neuromuscular Disorders Respiratory Complications and AssessmentNahid Sherbini
 
Congenital myasthenic syndrome
Congenital myasthenic syndromeCongenital myasthenic syndrome
Congenital myasthenic syndromePrashant Makhija
 
Guillain–Barré syndrome
Guillain–Barré syndromeGuillain–Barré syndrome
Guillain–Barré syndromebelton Mybelton
 
Ahd neuro-opthalmology - v. patel - nystagmus (1)
Ahd   neuro-opthalmology - v. patel - nystagmus (1)Ahd   neuro-opthalmology - v. patel - nystagmus (1)
Ahd neuro-opthalmology - v. patel - nystagmus (1)Ram Gopal
 
6 microbio (respiratory diseases)
6 microbio (respiratory diseases)6 microbio (respiratory diseases)
6 microbio (respiratory diseases)Merlyn Denesia
 
Complication of long term ventilation
Complication of long term ventilationComplication of long term ventilation
Complication of long term ventilationsaman priyantha
 
Salon 1 15 kasim 09.30 10.30 vedran dumbovi̇c
Salon 1 15 kasim 09.30 10.30 vedran dumbovi̇cSalon 1 15 kasim 09.30 10.30 vedran dumbovi̇c
Salon 1 15 kasim 09.30 10.30 vedran dumbovi̇ctyfngnc
 
Nm disorder
Nm disorderNm disorder
Nm disorderEM OMSB
 

Andere mochten auch (20)

Neuromuscular Diseases
Neuromuscular DiseasesNeuromuscular Diseases
Neuromuscular Diseases
 
Neuromuscular Disorders affecting the orofacial region
Neuromuscular Disorders affecting the orofacial regionNeuromuscular Disorders affecting the orofacial region
Neuromuscular Disorders affecting the orofacial region
 
Neuromuscular Disease
Neuromuscular DiseaseNeuromuscular Disease
Neuromuscular Disease
 
Neuromuscular Disorders
Neuromuscular DisordersNeuromuscular Disorders
Neuromuscular Disorders
 
Neuromuscular Junction Disease
Neuromuscular Junction DiseaseNeuromuscular Junction Disease
Neuromuscular Junction Disease
 
Evaluation and investigation of Neuromuscular disorders
Evaluation and investigation of Neuromuscular disordersEvaluation and investigation of Neuromuscular disorders
Evaluation and investigation of Neuromuscular disorders
 
Neuromuscular disorders for dental management
Neuromuscular disorders for dental managementNeuromuscular disorders for dental management
Neuromuscular disorders for dental management
 
Neuromuscular disorders in children (2)
Neuromuscular disorders in children (2)Neuromuscular disorders in children (2)
Neuromuscular disorders in children (2)
 
Lac mac and pac
Lac mac and pacLac mac and pac
Lac mac and pac
 
PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...
PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...
PATHOLOGICAL INVESTIGATIONS AND IMAGING TECHNIQUES IN NEUROMUSCULAR DISORDERS...
 
Pediatrics 5th year, 13th lecture/part one (Dr. Adnan)
Pediatrics 5th year, 13th lecture/part one (Dr. Adnan)Pediatrics 5th year, 13th lecture/part one (Dr. Adnan)
Pediatrics 5th year, 13th lecture/part one (Dr. Adnan)
 
Neuromuscular Disorders Respiratory Complications and Assessment
Neuromuscular Disorders Respiratory Complications and AssessmentNeuromuscular Disorders Respiratory Complications and Assessment
Neuromuscular Disorders Respiratory Complications and Assessment
 
Congenital myasthenic syndrome
Congenital myasthenic syndromeCongenital myasthenic syndrome
Congenital myasthenic syndrome
 
Guillain–Barré syndrome
Guillain–Barré syndromeGuillain–Barré syndrome
Guillain–Barré syndrome
 
Ahd neuro-opthalmology - v. patel - nystagmus (1)
Ahd   neuro-opthalmology - v. patel - nystagmus (1)Ahd   neuro-opthalmology - v. patel - nystagmus (1)
Ahd neuro-opthalmology - v. patel - nystagmus (1)
 
Nystagmus01
Nystagmus01Nystagmus01
Nystagmus01
 
6 microbio (respiratory diseases)
6 microbio (respiratory diseases)6 microbio (respiratory diseases)
6 microbio (respiratory diseases)
 
Complication of long term ventilation
Complication of long term ventilationComplication of long term ventilation
Complication of long term ventilation
 
Salon 1 15 kasim 09.30 10.30 vedran dumbovi̇c
Salon 1 15 kasim 09.30 10.30 vedran dumbovi̇cSalon 1 15 kasim 09.30 10.30 vedran dumbovi̇c
Salon 1 15 kasim 09.30 10.30 vedran dumbovi̇c
 
Nm disorder
Nm disorderNm disorder
Nm disorder
 

Ähnlich wie Neuromuscular disorder

Nervous system
Nervous systemNervous system
Nervous systemharneth
 
Central Nerves System
Central Nerves SystemCentral Nerves System
Central Nerves SystemUmarKhan68
 
Nerve supply of head & neck by Dr. Amit T. Suryawanshi, Oral Surgeon, Pune
Nerve supply of head & neck  by Dr. Amit T. Suryawanshi,  Oral Surgeon, Pune Nerve supply of head & neck  by Dr. Amit T. Suryawanshi,  Oral Surgeon, Pune
Nerve supply of head & neck by Dr. Amit T. Suryawanshi, Oral Surgeon, Pune All Good Things
 
Nerve supply of head & neck by Dr. Amit Suryawanshi .Oral & Maxillofacial ...
Nerve supply of head & neck   by  Dr. Amit Suryawanshi .Oral & Maxillofacial ...Nerve supply of head & neck   by  Dr. Amit Suryawanshi .Oral & Maxillofacial ...
Nerve supply of head & neck by Dr. Amit Suryawanshi .Oral & Maxillofacial ...All Good Things
 
L5 Muscle Structure
L5 Muscle StructureL5 Muscle Structure
L5 Muscle StructureMarc Potter
 
NERVOUS SYSSS
NERVOUS SYSSSNERVOUS SYSSS
NERVOUS SYSSSYanRecto2
 
Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...
Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...
Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...Shaista Jabeen
 
The Nervous System ppt.pdf
The Nervous System ppt.pdfThe Nervous System ppt.pdf
The Nervous System ppt.pdfAyeGob
 
Physiology of spinal cord by Tibbia college Delhi
Physiology of spinal cord by Tibbia college DelhiPhysiology of spinal cord by Tibbia college Delhi
Physiology of spinal cord by Tibbia college DelhiMudassirHussain21
 
Ch14 nervous tissue
Ch14 nervous tissueCh14 nervous tissue
Ch14 nervous tissueKemUnited
 
Organisation of ans
Organisation of ansOrganisation of ans
Organisation of ansSmita Jain
 
The Nervous SystemPPT.pptx
The Nervous SystemPPT.pptxThe Nervous SystemPPT.pptx
The Nervous SystemPPT.pptxDrNajimaParveen1
 
Anatomy-Nervous-System Anatomy and Physiology updated.pptx
Anatomy-Nervous-System Anatomy and Physiology updated.pptxAnatomy-Nervous-System Anatomy and Physiology updated.pptx
Anatomy-Nervous-System Anatomy and Physiology updated.pptxJRRolfNeuqelet
 
The nervous system
The nervous systemThe nervous system
The nervous systemMukul Kumar
 
Nervous System Vs PNS
Nervous System Vs PNSNervous System Vs PNS
Nervous System Vs PNSSonia Sanchez
 
Nervous System
Nervous SystemNervous System
Nervous System000 07
 

Ähnlich wie Neuromuscular disorder (20)

Nervous system
Nervous systemNervous system
Nervous system
 
Central Nerves System
Central Nerves SystemCentral Nerves System
Central Nerves System
 
Nervous system
Nervous systemNervous system
Nervous system
 
Nerve supply of head & neck by Dr. Amit T. Suryawanshi, Oral Surgeon, Pune
Nerve supply of head & neck  by Dr. Amit T. Suryawanshi,  Oral Surgeon, Pune Nerve supply of head & neck  by Dr. Amit T. Suryawanshi,  Oral Surgeon, Pune
Nerve supply of head & neck by Dr. Amit T. Suryawanshi, Oral Surgeon, Pune
 
Nerve supply of head & neck by Dr. Amit Suryawanshi .Oral & Maxillofacial ...
Nerve supply of head & neck   by  Dr. Amit Suryawanshi .Oral & Maxillofacial ...Nerve supply of head & neck   by  Dr. Amit Suryawanshi .Oral & Maxillofacial ...
Nerve supply of head & neck by Dr. Amit Suryawanshi .Oral & Maxillofacial ...
 
L5 Muscle Structure
L5 Muscle StructureL5 Muscle Structure
L5 Muscle Structure
 
NERVOUS SYSSS
NERVOUS SYSSSNERVOUS SYSSS
NERVOUS SYSSS
 
Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...
Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...
Nervous tissue types and functions, Neuron structure and types of Neurons, Ne...
 
The Nervous System ppt.pdf
The Nervous System ppt.pdfThe Nervous System ppt.pdf
The Nervous System ppt.pdf
 
Physiology of spinal cord by Tibbia college Delhi
Physiology of spinal cord by Tibbia college DelhiPhysiology of spinal cord by Tibbia college Delhi
Physiology of spinal cord by Tibbia college Delhi
 
Ch14 nervous tissue
Ch14 nervous tissueCh14 nervous tissue
Ch14 nervous tissue
 
Organisation of ans
Organisation of ansOrganisation of ans
Organisation of ans
 
The Nervous SystemPPT.pptx
The Nervous SystemPPT.pptxThe Nervous SystemPPT.pptx
The Nervous SystemPPT.pptx
 
Anatomy-Nervous-System Anatomy and Physiology updated.pptx
Anatomy-Nervous-System Anatomy and Physiology updated.pptxAnatomy-Nervous-System Anatomy and Physiology updated.pptx
Anatomy-Nervous-System Anatomy and Physiology updated.pptx
 
Neurological sysyem
Neurological sysyemNeurological sysyem
Neurological sysyem
 
The nervous system
The nervous systemThe nervous system
The nervous system
 
Nervous system ppt
Nervous system pptNervous system ppt
Nervous system ppt
 
Spinal Cord
Spinal CordSpinal Cord
Spinal Cord
 
Nervous System Vs PNS
Nervous System Vs PNSNervous System Vs PNS
Nervous System Vs PNS
 
Nervous System
Nervous SystemNervous System
Nervous System
 

Kürzlich hochgeladen

How to Add Existing Field in One2Many Tree View in Odoo 17
How to Add Existing Field in One2Many Tree View in Odoo 17How to Add Existing Field in One2Many Tree View in Odoo 17
How to Add Existing Field in One2Many Tree View in Odoo 17Celine George
 
Philosophy of Education and Educational Philosophy
Philosophy of Education  and Educational PhilosophyPhilosophy of Education  and Educational Philosophy
Philosophy of Education and Educational PhilosophyShuvankar Madhu
 
Presentation on the Basics of Writing. Writing a Paragraph
Presentation on the Basics of Writing. Writing a ParagraphPresentation on the Basics of Writing. Writing a Paragraph
Presentation on the Basics of Writing. Writing a ParagraphNetziValdelomar1
 
CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...
CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...
CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...Nguyen Thanh Tu Collection
 
Diploma in Nursing Admission Test Question Solution 2023.pdf
Diploma in Nursing Admission Test Question Solution 2023.pdfDiploma in Nursing Admission Test Question Solution 2023.pdf
Diploma in Nursing Admission Test Question Solution 2023.pdfMohonDas
 
How to Show Error_Warning Messages in Odoo 17
How to Show Error_Warning Messages in Odoo 17How to Show Error_Warning Messages in Odoo 17
How to Show Error_Warning Messages in Odoo 17Celine George
 
Patient Counselling. Definition of patient counseling; steps involved in pati...
Patient Counselling. Definition of patient counseling; steps involved in pati...Patient Counselling. Definition of patient counseling; steps involved in pati...
Patient Counselling. Definition of patient counseling; steps involved in pati...raviapr7
 
The Stolen Bacillus by Herbert George Wells
The Stolen Bacillus by Herbert George WellsThe Stolen Bacillus by Herbert George Wells
The Stolen Bacillus by Herbert George WellsEugene Lysak
 
Practical Research 1: Lesson 8 Writing the Thesis Statement.pptx
Practical Research 1: Lesson 8 Writing the Thesis Statement.pptxPractical Research 1: Lesson 8 Writing the Thesis Statement.pptx
Practical Research 1: Lesson 8 Writing the Thesis Statement.pptxKatherine Villaluna
 
How to Manage Cross-Selling in Odoo 17 Sales
How to Manage Cross-Selling in Odoo 17 SalesHow to Manage Cross-Selling in Odoo 17 Sales
How to Manage Cross-Selling in Odoo 17 SalesCeline George
 
UKCGE Parental Leave Discussion March 2024
UKCGE Parental Leave Discussion March 2024UKCGE Parental Leave Discussion March 2024
UKCGE Parental Leave Discussion March 2024UKCGE
 
P4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdf
P4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdfP4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdf
P4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdfYu Kanazawa / Osaka University
 
Practical Research 1 Lesson 9 Scope and delimitation.pptx
Practical Research 1 Lesson 9 Scope and delimitation.pptxPractical Research 1 Lesson 9 Scope and delimitation.pptx
Practical Research 1 Lesson 9 Scope and delimitation.pptxKatherine Villaluna
 
Quality Assurance_GOOD LABORATORY PRACTICE
Quality Assurance_GOOD LABORATORY PRACTICEQuality Assurance_GOOD LABORATORY PRACTICE
Quality Assurance_GOOD LABORATORY PRACTICESayali Powar
 
AUDIENCE THEORY -- FANDOM -- JENKINS.pptx
AUDIENCE THEORY -- FANDOM -- JENKINS.pptxAUDIENCE THEORY -- FANDOM -- JENKINS.pptx
AUDIENCE THEORY -- FANDOM -- JENKINS.pptxiammrhaywood
 
Maximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdf
Maximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdfMaximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdf
Maximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdfTechSoup
 
General views of Histopathology and step
General views of Histopathology and stepGeneral views of Histopathology and step
General views of Histopathology and stepobaje godwin sunday
 

Kürzlich hochgeladen (20)

How to Add Existing Field in One2Many Tree View in Odoo 17
How to Add Existing Field in One2Many Tree View in Odoo 17How to Add Existing Field in One2Many Tree View in Odoo 17
How to Add Existing Field in One2Many Tree View in Odoo 17
 
Prelims of Kant get Marx 2.0: a general politics quiz
Prelims of Kant get Marx 2.0: a general politics quizPrelims of Kant get Marx 2.0: a general politics quiz
Prelims of Kant get Marx 2.0: a general politics quiz
 
Philosophy of Education and Educational Philosophy
Philosophy of Education  and Educational PhilosophyPhilosophy of Education  and Educational Philosophy
Philosophy of Education and Educational Philosophy
 
Presentation on the Basics of Writing. Writing a Paragraph
Presentation on the Basics of Writing. Writing a ParagraphPresentation on the Basics of Writing. Writing a Paragraph
Presentation on the Basics of Writing. Writing a Paragraph
 
CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...
CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...
CHUYÊN ĐỀ DẠY THÊM TIẾNG ANH LỚP 11 - GLOBAL SUCCESS - NĂM HỌC 2023-2024 - HK...
 
Diploma in Nursing Admission Test Question Solution 2023.pdf
Diploma in Nursing Admission Test Question Solution 2023.pdfDiploma in Nursing Admission Test Question Solution 2023.pdf
Diploma in Nursing Admission Test Question Solution 2023.pdf
 
How to Show Error_Warning Messages in Odoo 17
How to Show Error_Warning Messages in Odoo 17How to Show Error_Warning Messages in Odoo 17
How to Show Error_Warning Messages in Odoo 17
 
Patient Counselling. Definition of patient counseling; steps involved in pati...
Patient Counselling. Definition of patient counseling; steps involved in pati...Patient Counselling. Definition of patient counseling; steps involved in pati...
Patient Counselling. Definition of patient counseling; steps involved in pati...
 
The Stolen Bacillus by Herbert George Wells
The Stolen Bacillus by Herbert George WellsThe Stolen Bacillus by Herbert George Wells
The Stolen Bacillus by Herbert George Wells
 
Practical Research 1: Lesson 8 Writing the Thesis Statement.pptx
Practical Research 1: Lesson 8 Writing the Thesis Statement.pptxPractical Research 1: Lesson 8 Writing the Thesis Statement.pptx
Practical Research 1: Lesson 8 Writing the Thesis Statement.pptx
 
How to Manage Cross-Selling in Odoo 17 Sales
How to Manage Cross-Selling in Odoo 17 SalesHow to Manage Cross-Selling in Odoo 17 Sales
How to Manage Cross-Selling in Odoo 17 Sales
 
UKCGE Parental Leave Discussion March 2024
UKCGE Parental Leave Discussion March 2024UKCGE Parental Leave Discussion March 2024
UKCGE Parental Leave Discussion March 2024
 
Personal Resilience in Project Management 2 - TV Edit 1a.pdf
Personal Resilience in Project Management 2 - TV Edit 1a.pdfPersonal Resilience in Project Management 2 - TV Edit 1a.pdf
Personal Resilience in Project Management 2 - TV Edit 1a.pdf
 
Finals of Kant get Marx 2.0 : a general politics quiz
Finals of Kant get Marx 2.0 : a general politics quizFinals of Kant get Marx 2.0 : a general politics quiz
Finals of Kant get Marx 2.0 : a general politics quiz
 
P4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdf
P4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdfP4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdf
P4C x ELT = P4ELT: Its Theoretical Background (Kanazawa, 2024 March).pdf
 
Practical Research 1 Lesson 9 Scope and delimitation.pptx
Practical Research 1 Lesson 9 Scope and delimitation.pptxPractical Research 1 Lesson 9 Scope and delimitation.pptx
Practical Research 1 Lesson 9 Scope and delimitation.pptx
 
Quality Assurance_GOOD LABORATORY PRACTICE
Quality Assurance_GOOD LABORATORY PRACTICEQuality Assurance_GOOD LABORATORY PRACTICE
Quality Assurance_GOOD LABORATORY PRACTICE
 
AUDIENCE THEORY -- FANDOM -- JENKINS.pptx
AUDIENCE THEORY -- FANDOM -- JENKINS.pptxAUDIENCE THEORY -- FANDOM -- JENKINS.pptx
AUDIENCE THEORY -- FANDOM -- JENKINS.pptx
 
Maximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdf
Maximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdfMaximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdf
Maximizing Impact_ Nonprofit Website Planning, Budgeting, and Design.pdf
 
General views of Histopathology and step
General views of Histopathology and stepGeneral views of Histopathology and step
General views of Histopathology and step
 

Neuromuscular disorder

  • 2. The Neuron  Defining unit of the nervous system  Specialized cell of the nervous system  Consists of a cell body, 5–25 µm in diameter, with branching processes (dendrites) that are capable of receiving signals from other neuronal terminals  A finer, longer branch (the axon) carries the action potentials along its length to or from excitable target organs  Further signal transmission to the dendrites of another neuron, or neuro-excitable tissue like muscle, occurs at a synapse where the axon terminal releases a chemical neurotransmitter – typically acetylcholine
  • 3.  All motor axons and the larger sensory axons serving touch, pain and proprioception are covered by a sheath – the neurilemma – and coated with myelin, a multilayered lipoprotein substance derived from the accompanying Schwann cells (or oligodendrocytes in the central nervous system).
  • 4.  Every few millimetres the myelin sheath is interrupted, leaving short segments of bare axon called the nodes of Ranvier.  In these nerves the myelin coating serves as an insulator, which allows the impulse to be propagated by electromagnetic conduction from node to node, much faster than is the case in unmyelinated nerves.  Consequently, depletion of the myelin sheath causes slowing – and eventually complete blocking – of axonal conduction
  • 5.  Most axons, in particular the small- diameter fibres carrying crude sensation and efferent sympathetic fibres, are not myelinated but wrapped in Schwann cell cytoplasm.  Damage to these axons causes unpleasant or bizarre sensations and abnormal sudomotor and vasomotor effects.
  • 6. NERVOUS PATHWAYS  Anatomically, neurological structures can be divided into the central nervous system (the CNS, comprising the brain and tracts of the spinal cord) and the peripheral nervous system (PNS) which includes the cranial and spinal nerves  In terms of physiological function, both the CNS and the PNS have a somatic component and an autonomic component
  • 7.  The somatic nervous system provides efferent motor and afferent sensory pathways to and from peripheral parts of the body serving, respectively, voluntary muscle contraction and sensibility  The autonomic system controls involuntary reflex and homeostatic activities of the cardiovascular system, visceral organs and glands. Its two components, sympathetic and parasympathetic divisions, serve more or less opposing functions.
  • 8. CNS and PNS  Central nervous system consists of the brain and spinal cord  Peripheral nervous system constitutes the link between the CNS and structures in the periphery of the body, from which it receives sensory information and to which it sends controlling impulses  T he peripheral nervous system consists of nerves joined to the brain and spinal cord (cranial and spinal nerves) and their ramifications within the body
  • 9. Main nerve pathways Simplified diagram showing the main neurological pathways to and from a typical thoracic spinal cord segment. Fibres carrying touch, sharp pain and temperature impulses (-------) decussate, in some cases over several spinal segments, and ascend in the contralateral spinothalamic tracts; those carrying vibration and proprioceptive impulses (——) enter the ipsilateral posterior columns. Motor neurons (——) arise in the anterior horn of the grey matter and innervate ipsilateral muscles.
  • 11. SOMATIC MOTOR SYSTEM  Efferent impulses are conducted along axons in the corticospinal or pyramidal tracts (upper motor neurons – UMN) and along peripheral nerves from cell bodies in the anterior horn of the spinal cord to striated muscle fibres (lower motor neurons – LMN)  The terminal synapses are situated at the neuromuscular junctions.
  • 12.  Each large a-motor neuron innervates from a few to several hundred muscle fibres (together forming a motor unit) and stimulates muscle fibre contraction.  In large muscles of the lower limb, power is adjusted by recruiting more or fewer motor units.  Smaller γ-motor neurons connect to sensors (muscle spindles) that control proprioceptive feedback from muscle fibres.
  • 14. SOMATIC SENSORY SYSTEM  Axons conveying afferent impulses from receptors in the skin and other peripheral structures enter the dorsal nerve roots, with their cell bodies in the dorsal root (or cranial nerve) ganglia, and end in synapses within the central nervous system.  Myelinated fibres carrying sensory stimuli from touch, pressure, pain and temperature (exteroceptive sensation) decussate and enter the contralateral spinothalamic tracts running up the spinal cord to the brain.
  • 15.  Fibres from sensors in the joints, ligaments, tendons and muscle carrying the sense of movement and bodily position in space (proprioceptive sensation) join the ipsilateral posterior columns in the spinal cord.
  • 18. REFLEX ACTIVITY AND TONE  Sudden stretching of a muscle (e.g. by tapping sharply over the tendon) induces an involuntary muscle contraction – the stretch reflex  The sharp change in muscle fibre length is detected by the muscle spindle; the impulse is transmitted rapidly along myelinated afferent (sensory) neurons which synapse directly with the corresponding segmental α-motor neurons in the spinal cord, triggering efferent signals which stimulate the muscle to contract.
  • 20.  Segmental reflex activity is normally regulated by motor impulses passing from the brain down the spinal cord.  Interruption of the UMN pathways results in undamped reflex muscle contraction (clinically hyperactive tendon reflexes) and spastic paralysis.  Damage to either afferent or efferent neurons in the reflex arc causes hypotonia; interruption of the LMN pathway results in flaccid LMN paralysis.
  • 21. AUTONOMIC SYSTEM  The autonomic system is involved with the regulation of involuntary activities of cardiac muscle and smooth (unstriated) muscle of the lungs, gastrointestinal tract, kidneys, bladder, genital organs, sweat glands and small blood vessels, with afferent (sensory) and efferent (motor) pathways constituting a continuously active reflex arc (though there is also some input from higher centres).  In addition afferent fibres also convey visceral pain sensation.
  • 22.  Preganglionic sympathetic neurons leave the spinal cord with the ventral nerve roots at all levels from T1 to L1, enter the paravertebral sympathetic chain of ganglia and synapse with postganglionic neurons that spread out to all parts of the body; they may also run up or down the sympathetic chain to synapse in other ganglia or pass on to become splanchnic nerves  Important functions are the reflex control of heart rate, blood flow and sweating, as well as other responses associated with conditions of ‘fight and flight’.
  • 24.  Parasympathetic neurons leave the CNS (from the brain-stem) with cranial nerves III, VII, IX, X and with the nerve roots of S2, 3 and 4 to reach ganglia where they synapse with postganglionic neurons close to their target organs.
  • 26. Peripheral nerves  Peripheral nerves are bundles of axons conducting efferent (motor) impulses from cells in the anterior horn of the spinal cord to the muscles, and afferent (sensory) impulses from peripheral receptors via cells in the posterior root ganglia to the cord.  They also convey sudomotor and vasomotor fibres from ganglion cells in the sympathetic chain.
  • 29. SKELETAL MUSCLE  Each skeletal muscle belly, held within a connective tissue epimysium, consists of thousands of muscle fibres, separated into bundles (or fascicles).  Each fascicle is surrounded by a flimsy perimysium which envelops anything up to about 100 muscle fibres; large muscles concerned with mass movement, like the glutei or quadriceps, have a large number of fibres in each fascicle, while muscles used for precision movements (like those of the hand) have a much smaller number in each bundle.
  • 30.  The muscle fibre is the important unit of all striated muscle. Lying in a barely discernable connective tissue cover, or endomysium, it is in actuality a single cell with a cell membrane (the sarcolemma), a type of cytoplasm (or sarcoplasm), mitochondria and many thousands of nuclei; its diameter is about 10 µm at birth and 60–80 µm in mature adults
  • 31.  The α-motor neuron and the group of muscle fibres it supplies constitute a single motor unit; the number of muscle fibres in the unit may be less than five in muscles concerned with fine manipulatory movements or more than 100 in those employed in gross power movements.
  • 33. Muscle Fibre  Muscle fibres are also of different types, which can be distinguished by histochemical staining.  Type I fibres contract slowly and are not easily fatigued; their prime function is postural control.  Type II fibres are fast contracting but they fatigue rapidly; hence they are ideally suited to intense activities of short duration.
  • 34.  All muscles consist of a mixture of fibre types, the balance depending on anatomical site, basic muscle function, degree of training, genetic disposition and response to previous injury or illness.  Long-distance runners have a greater proportion of type I fibres than the average in age- and sex-matched individuals
  • 36. Muscle Contraction  Muscle contraction is a complex activity. Individual myofibrils respond to electrical stimuli in much the same way as do motor neurons. However, muscle fibres, and the muscle as a whole, are activated by overlap and summation of contractile responses.  When the fibres contract, internal tension in the muscle increases.
  • 40.  In isometric contraction there is increased tension without actual shortening of the muscle or movement of the joint controlled by that muscle.  In isotonic contraction the muscle shortens and moves the joint, but tension within the muscle fibres remains constant.
  • 41. Muscle Tone  Muscle tone is the state of tension in a resting muscle when it is passively stretched; characteristically tone is increased in upper motor neuron (UMN) lesions (spastic paralysis) and decreased in lower motor neuron (LMN) lesions (flaccid paralysis).
  • 42. Muscle contracture  Muscle contracture (as distinct from contraction) is the adaptive change which occurs when a normally innervated muscle is held immobile in a shortened position for some length of time. If a joint is allowed to be held flexed for a long time, it may be impossible to straighten it passively without injuring the muscle.  Active exercise will eventually overcome the muscle contracture, unless the muscle has been permanently damaged.
  • 43. Muscle Wasting  follows either disuse or denervation; in the former, the fibres are intact but thinner; in the latter, they degenerate and are replaced by fibrous tissue or fat
  • 44. Muscle Fasciculation  Muscle fasciculation – or muscle twitch – is a local involuntary muscle contraction of a small bundle of muscle fibres.  It is usually benign but can be due to motor neuron disease or dysfunction.
  • 46. History Age  important  Arthrogryphosis & spina bifida  at birth  Cerebral palsy -> later in childhood  Poliomyelitis  childhood  may be seen in any age  Spinal cord lesions & peripheral neuropathies  common in adults  Orthopaedic surgeon  mainly with residual effects of neurological disease  may require diagnosis & treatment throughout life
  • 47. Muscle weakness : UMN, LMN or muscle disorders Type of weakness, distribution, rate of onset  diagnosis Numbness & paraesthesiae : May be main complaints Important to establish their exact distribution  localize lesion accurately Rate of onset & relationship to posture  cause
  • 48. Deformity  Common complaint in long-standing disorders  From muscle imbalance  hand in hand with other symptoms  Minor degrees of weakness in 1 muscle group may unnoticed  deformity appears so insidiously  may escape detection  eg. claw toes, scoliosis
  • 49. Other Features  Headache  Dizziness  Loss of balance  Change in visual acuity / hearing  Disorder of speech  Loss of bladder / bowel control
  • 50. Examination  Complete neurological assessment  The back  skin changes, local deformities, mobility Patient’s mental state Muscle tone & power Natural posture Reflexes Gait Skin changes Sense of balance Various modes of sensibility & autonomic functions (eg. sphincter control) Involuntary movements Peripheral blood flow Muscle wasting Sweating
  • 51. Grading Muscle Power  Repetition  progress to be recorded 0 Total paralysis 1 Barely detectable contracture 2 Not enough power to act against gravity 3 Strong enough to act against gravity 4 Still stronger but less than normal 5 Full power
  • 55. GAIT and POSTURE  A single gait cycle consists of a stance phase (60 per cent) and a swing phase (40 per cent) and each full cycle represents the stride length  Dystonia – This term refers to abnormal posturing (focal or generalized) that may affect any part of the body and is often aggravated when the patient is concentrating on a particular motor task such as walking
  • 63. Motor Power and Tone GRADE DESCRIPTION 0 No muscle action. Total paralysis 1 Minimal muscle contraction 2 Power insufficient to overcome gravity 3 Anti-gravity muscle power 4 Less than full power 5 Full power
  • 64. Weakness  Monoplegia  Indicative for lower motor neuron defect  Movement affected on clinical test will suggest the anatomical location  Hemiparesis  Weakness either the right or left side  Pathology between cerebral cortex and cervical segment of spinal cord  Upper Motor Neuron type (spastic)  Complete loss of power: hemiplegia
  • 65. Weakness  Diplegia  Both upper limb or both lower limb  Can be either UMN or LMN disorder  Quadriplegia  All four limb affected
  • 66. Deformity  Unbalanced paralysis  One group of muscle is too weak to balance the pull of the antagonis  Balanced paralysis  The joint assumed that the position imposed on it by gravity and it may feel floppy or flail
  • 68. Imaging  Plain X-ray  Routine for all disorders  Fracture and dislocation  CT-Scan  Reveal relation between bone fragment to nerve structure  MRI
  • 69. NEUROPHYSIOLOGICAL STUDIES Motor Nerve Conduction  Stimulate electrically at an easy subcutaneous site until it propagates an action potensial on target muscle
  • 70. Measurement:  Latency  It takes in ms (millisecond)  Time for impulse to reach the muscle  Amplitude of the Compound Muscle Action Potential (CMAP)  In mV (millivolts)  Magnitude of the response  Nerve Conduction Velocity  Measure the distance from stimulating electrode to the recording elecctrode, and divide by the latency
  • 71.  ms  time for impulse to reach muscle  latency  mV  magnitude of response  amplitude of the evoked compound muscle action potential (CMAP)  By measuring the distance from the stimulating electrode to recording electrode, and setting against latency  nerve conduction velocity (NCV)  metres/second  In practice, more useful & accurate to stimulate the nerve at 2 points  distal & proximal site, and subtract distal latency from proximal latency to obtain a truer measurement for intervening segment of nerve
  • 72.  To measure NCV of median nerve in carpal tunnel  stimulating electrode first distal to carpal tunnel & then in upper forearm  Amplitude  proportional to number of motor units stimulated : if patient has lost ½ of nerve fibres in peripheral nerves (compression, trauma, vascular insufficiency), size of elicited CMAP will be reduced by ±50% compared to contralateral normal limb  CMAP on proximal stimulation smaller than distal stimulation  conduction block  a feature of a potentially recoverable neuropraxic lesion
  • 73. Conduction slowing of uniform degree along the whole length of nerve  demyelinating neuropathy  Charcot – Marie – Tooth syndrome
  • 74. Sensory Nerve Conduction In a similar manner, sensory nerve action potential (SNAP) may be recorded by stimulating a suitable subcutaneous sensory nerve & recording with surface electrodes on the skin over a measured distance along the same sensory nerve  from index & middle fingers of median nerve SNAP is much smaller in amplitude than CMAP  microvolts
  • 75. Clinical nerve conduction studies estimate population of large myelinated sensory or motor nerves Type C fibres (small myelinated fibres  pain & temperature)  amplitude below sensitivity of recording techniques & slowed velocity (5-10 m/sec)  cannot be tested with standard clinical techniques
  • 77. Electromyography (EMG)  Concentric needle electrode (small hypodermic needle) is inserted into muscle & connected to oscilloscopic screen & loudspeaker  record electrical discharge of motor units in a muscle  visual pattern & crackling sounds  At rest, normal muscle is silent  Patient slowly contracts  progressive  in number & also amplitude of motor unit action potentials  recognizable pattern
  • 78. Full recruitment pattern usually looks & sounds like ‘white noise’  so many motor units firing  both spikes on screen & crackles from speakers overlap each other  interference pattern In nerve disorders, muscle may not be silent at rest   insertional activity
  • 79. Motor nerve fibre loss / disruption  changes of active denervation (fibrillation potentials & positive sharp waves)  denervated muscle fibres firing spontaneously  7-12 days after axonal disruption
  • 80. Chronic neuropathy, with re-sprouting of remaining viable nerve fibres  longer re-innervated motor units with polyphasic or higher amplitude profile
  • 81. Diagnostic Evaluation of The Patient  When investigating a specific nerve root syndrome, nerve conduction & EMG studies are concentrated in appropriate anatomical territory  findings are compared to those in other nerve root territories in the same level as well as the contralateral (usually asymptomatic) limb  Mononeuropathy / plexopathy  compare conduction values (amplitude & velocity) in 1 limb to those in the other
  • 82.  Focal entrapment  reduced amplitude on proximal stimulation compared to distal stimulation  conduction block or significant focal conduction slowing  Neurophysiological signs of neuropathic disorder :   motor or sensory potentials  nonfunctioning (perhaps transected) nerves  Loss of sensory responses (SNAP)  disorder distal to spinal foramen; intact SNAP in hypaesthetic limb  disease proximal to foramen
  • 83.  Conduction block  neuropraxic recoverable injury  Denervation changes on EMG >10 days after injury  significant nerve damage & loss of motor nerve function  Any recruited volitional motor units in a weak limb  potential for recovery  The presence of intact sensory potential is what distinguishes root & proximal disease from peripheral entrapment & plexus disease
  • 84. Intraoperative Neurophysiological Techniques Spinal Monitoring : somatosensory evoked responses (SSEP)  Neurophysiological tests are sometimes necessary during corrective spinal operations  obviate injury to the cord  EEG  averaging  records from scalp overlying patient’s sensory parietal cortex  one must average the obtained responses from at least 100-200 stimuli to differentiate time- linked evoked response from the background brain EEG activity
  • 85.  The important measured parameter is usually the latency of the response  Accidental nerve injury during surgery around spinal cord will produce a delay in the latency or a sudden loss of the evoked response  Other intraoperative techniques :  Nerve or nerve root stimulation  demonstrate conduction block or slowing or normal continuity of nerve  Intraoperative EMG  Cord-to-cord stimulation & cord-to-cortical potential measurement  reveal intraoperative evidence of spinal pathway disruption
  • 88. Examination A group of disorders result from non-progressive brain damage during early development  abnormal movement & posture 2 : 1000 live births  highest in premature babies & multiple births Causes : maternal toxaemia, prematurity, perinatal anoxia, kernicterus, postnatal brain infections / injury Birth injury  unusual cause
  • 89.  May also cause damage to other areas of developing brain  epilepsy, perceptual & behavioural problems, learning difficulties  Main consequence  development of neuromuscular incoordination, dystonia, weakness, spasticity  Oro-facial motor incoordination  difficult speech & swallowing, drooling  None of these defects implies poor intellect
  • 90. Classification  Usually according to type of motor disorder, with subdivisions referring to topographical distribution of clinical signs
  • 91. Type of Motor Disorder Spasticity  commonest  damage to pyramidal system in CNS   muscle tone & hyper-reflexia Resistance to passive movement may obscure a basic weakness of affected muscles Hypotonia  a phase  several years during early childhood before features of spasticity become obvious
  • 92. Athetosis  continuous, involuntary, writhing movements  damage to extrapyramidal systems of CNS Pure athetoid CP  joint contractures are unusual, muscle tone is not  Dystonia  may occur with athetosis  more generalized  in muscle tone & abnormal positions induced by activity
  • 93. Ataxia  muscular incoordination during voluntary movements  due to cerebellar damage  balance is poor  walks with a characteristic wide-based gait Mixed palsy  combination of spasticity & athetosis  can make results of surgical intervention unpredictable
  • 97.  In some types of CP  considerable variability in ‘tone’ & ‘posture’ from day to day / situation to situation  If surgical treatment being considered, never based on a single assessment when, due to stress, child appears to have abnormally high tone & muscle contractures
  • 101. Topographic Distribution  Hemiplegia  commonest  spastic palsy on 1 side of body with upper limb more severely affected than lower  most can walk & respond reasonably well to treatment  Diplegia  both sides of body with lower limbs always most severely affected Side to side involvement may be asymmetrical  asymmetric diplegia, bilateral hemiplegia Many cases are secondary to prematurity & periventricular leucomalacia on brain MRI Intelligence is often normal Less severely  reasonable mobility
  • 102.  Total body involvement  general & often more severe disorder affecting all 4 limbs, trunk, neck, face with varying degrees of severity Usually have low IQ, may have epilepsy, often unable to walk, poor treatment response
  • 103. Monoplegia  occasionally in an upper limb, other areas are involved as well True monoplegia  so unusual  other diagnoses should be considered (eg. Neonatal brachial plexopathy)
  • 105. Diagnosis in Infancy  Full-blown clinical picture may take months / years to develop  Prenatal toxaemia, haemorrhage, premature birth, difficult labour, foetal distress, kernicterus  arouse suspicion  Neonatal ultrasound scan of head  intracerebral bleeding
  • 106.  Early symptoms:  Difficulty in sucking & swallowing, dribbling at mouth  Baby feels stiff / wriggles awkwardly  Apparent that motor milestones are delayed  Normal : holds up its head at 3 mo, sits up at 6 mo, begins walking at 1 year
  • 107. Diagnosis in Later Childhood  Bleck (1987)  7 tests for children over 1 year  idea of severity & prognosis for walking  Primitive neck-righting reflex, asymmetrical & symmetrical tonic neck reflexes, Moro reflex, extensor thrust response  all disappeared at 1 year  Retain > 2 primitive reflexes, can’t sit unsupported by 4 yo, can’t walk unaided by 8 yo  unlikely ever to walk independently
  • 108. Ideally reviewed by a multidisciplinary team Gross Motor Function Classification System (GMFCS)  relative to their age, in terms of mobility & bases this on their average function, not the best that they can achieve on a given occasion  reliable & valid
  • 110. Sitting Posture  Children with a hypotonic trunk may slump into a kyphotic posture & others may always ‘fall’ to one side  In attempting to sit, lower limbs may be thrust into extension  May be an obvious scoliosis / pelvis obliquity
  • 111. Standing Posture  Typical case of spastic diplegia  stands with hips flexed, adducted, internally rotated; knees flexed; feet equinus  With tight hamstrings, normal lumbar lordosis may be obliterated & may have difficulty standing unsupported  Often attempts to correct 1 deformity may aggravate another  important to establish which deformity are primary & compensatory
  • 112. Many patients show pelvic obliquity & scoliosis Asking child to ‘stand tall’, watching their response often gives some insight into dynamic nature of posture & muscle strength, intellectual ability Balance reactions are often poor
  • 114. Gait  Observed with & w/o shoes / orthotic supports  Dystonic, athetoid, ataxic movements may become more noticeable during walking  Every opportunity must be taken to observe gait  differences between ‘normal’ & ‘best behaviour’ walking can be identified  In hemiplegics, best behaviour walking may demonstrate a flat foot pattern with heel coming down most of the time while more normal / representative pattern will highlight asymmetric flexed knee & toe- walking pattern
  • 115. Clinical Gait Analysis  Each limb must be observed in both stance & swing phases of gait & in coronal, sagittal & transverse planes  Lack of free rotation at hip  trunk has to move from side to side as each leg swings through & with adduction  ‘scissoring’ action  Narrow walking base, when combined with hip & knee flexion & foot equinus  strong tendency to fall  helped by cruthces
  • 116. Computerized Gait Analysis  Ideally supplements observational gait analysis  Kinematics (joint & limb segment movement)  Kinetics (joint moments & powers)  EMG (identification of phases in which muscles are firing)  Pedobarography (foot pressures)  Metabolic energy analysis (assessment of ‘cost’ of walking) • To help clinician distinguish between dynamic & fixed tightness & in identification of dyskinesia
  • 117. Neuromuscular Examination  Typical features of UMN  Muscle tone, power & ROM at each joint  Physical signs may vary from day to day / even minute to minute  emotional state, room temperature  Takes time
  • 118. Deformity Assessment  At each joint & relate it to muscle-tendon length  Deformity at 1 level may be markedly affected by position of joints above & below  Ankle equinus with knee extended often disappears when knee is flexed  can differentiate between tightness in soleus & gastrocnemius muscle
  • 119. Silfverskiöld Test  Supine on examination couch  Knee flexed to a right angle & ankle dorsiflexed  tests soleus tightness  Then knee fully extended & ankle dorsiflexion is repeated  tests gastrocnemius tightness  Tight hamstrings may limit knee extension more with hips flexed than when hips extended  Tight gracilis  hip adduction may be easier in flexion than in extension
  • 121. Hip abduction is restricted  order x-ray to look for subluxation of joint In upper limb, finger flexors may be tight with wrist extended but if wrist is allowed to flex the fingers can extend Children can use these fixed-length reactions to manipulate their hand & finger function using ‘trick’ movements
  • 122.  Patient with total body involvement  spinal deformity is common  scoliosis, often associated with pelvic obliquity  Kyphosis & lordosis also occur SENSATION  Often not entirely normal  Problems with stereognosis (as well as with perception)  important factors contributing to upper limb disability
  • 123. Muscle Contracture  A degree of muscle contracture is almost inevitable with all forms of CP  longstanding spasticity  relative shortening of muscles  fixed contractures & changes in joint congruity  Most of the effects seen during period of growth  After skeletal maturity, changes in muscle-tendon length & joint contracture much less progressive
  • 124. Bony Deformity  Normal bone growth is influenced by muscle pull  Children with persistent abnormal muscle pull  failure of normal modelling & new deformities can develop  Normal degree of femoral neck anteversion persists & sometimes even increases with growth rather than improving  significant external tibial torsion may also be present
  • 125. Persistent adduction of hip  valgus of femoral neck, acetabular dysplasia, subluxation of joint Flexion deformity of knee  upward displacement of patella & patello-femoral pain External tibial torsion  planovalgus deformity of foot
  • 126. Structural Scoliosis Flexible curves are common, but many become structural  especially likely in total body involvement
  • 128. Management No single ‘blueprint’ Goal Setting Few patients with total body involvement will ever talk Prognosis for walking in spastic diplegia  Bleck’s criteria & Beals
  • 129. Priorities for all CP patients are : Ability to communicate with others Ability to cope with activities of daily living (including personal hygiene) Independent mobility  may mean a motorized wheelchair rather than walking
  • 130. Realistic goals for child who from an early age is recognized to be ‘non-walking’ are : Straight spine with a level pelvis Located, mobile, painless hip that flex 90o & extend  comfortable sleeping & participation in standing / swivel transfers Knees that mobile enough for sitting, sleeping, transferring Plantigrade feet that fit into shoes & rest on footplates of wheelchair comfortably
  • 131. Tone Management Medical treatment   anticonvulsants for seizures,  short-term benzodiazepine for postoperative pain,  trihexyphenadryl for dystonia
  • 132. Baclofen  Agonist gamma-aminobutryic acid (GABA)  inhibits reflex activity  Oral  doesn’t cross blood-brain barrier well  Reduces muscle tone / spasticity generally  Negative effect on head & trunk control  side effects : drowsiness  its use may be limited  Intrathecal  via refillable, subcutaneous implanted pump  dose administered can be titrated according to child’s response
  • 133. Long-term studies not yet available  appears most effective in severe spasticity / dystonia Not effective in all patients & test doses & assessment of its benefits required in all prospective patients
  • 134.  Dantrolene Produces weakness w/o much  in spasticity  rarely used in CP  Analgesic medication  pain associated with muskuloskeletal problems, constipation, gastro-oesophageal reflux
  • 135. Botulinum Toxin  Blocking acetyl choline release at neuromuscular junction  Injected into ‘spastic’ muscle at (or as near as possible to) motor end point  Usual targets : hip adductors, hamstrings, gastrocnemius, tibialis posterior  Weakness / paralysis takes a few days to become obvious  temporary (as new nerve terminals form)  10-12 wk
  • 136. Not be used on its own Followed by  physiotherapy input & often an alteration in orthotic / splinting regimens Focal treatment for a dynamic muscle imbalance that is interfering with function  deformity, pain More effective in younger children  less likely to have fixed deformity
  • 137. Multilevel injections may be required but overall dose per child must be kept within safe limits For postoperative pain & spasm  for optimal effect, need to be given some days prior to surgery
  • 138. Selective Dorsal Rhizotomy  Division of selected dorsal nerve roots from L1 to S2 has only recently gained wide acceptance   spasticity & rebalance muscle tone  selectively  input from muscle spindles  less excitation of anterior horn cells  Long-term studies not yet available
  • 139. Good results in children aged 3-8 years with criteria :  Walking but have significant spasticity  Born prematurely  Have good intellectual function & good voluntary control Relative contraindication : fixed contractures  may need surgical correction
  • 140. Physical Therapy   or prevent problems arising from abnormal muscle tone, imbalance between opposing muscle groups & abnormal body balance mechanisms  A range of regular movement exercises will prevent or (perhaps more realistically)  degree of muscle / joint contracture  Most helpful in early childhood up to age 7 or 8 years  Postoperative physiotherapy is essential  maximize effects of surgery & overcome immediate pain, stiffness & weakness
  • 141.  Positioning & splinting Disadvantageous positions  hip adduction  Splints To prevent muscle contracture, maintain joint position, improve movement & function, maintaining position following surgery Badly fitting splint  does nothing – provokes pain & spasm &  deformity  Manipulation & serial casting Limited role in improving muscle / joint contractures  relaps is frequent
  • 142. Operative Treatment Indications :  Spastic deformity which cannot be controlled by conservative measures  Fixed deformity that interferes with function  Secondary complications  bony deformities, dislocation of hip & joint instability Weak muscles can be augmented by tendon transfers  gravity plays important part in guiding choice of tendon transfers
  • 143. Timing is often crucial CNS & gait pattern matures around age 7-8 years Our preferred approach is to avoid ‘little and often’ surgery in favour of ‘all or none’ philosophy, but some patients require former & some the latter Earlier operation may be called for if hip threatens to dislocate
  • 144. Regional Survey : Upper Limb Most typically in child with spastic hemiplegia or total body involvement  flexion of elbow, pronation of forearm, flexion of wrist, clenched fingers, adduction of thumb Aimed at improving resting position of limb & restorating grasp
  • 145. Elbow Flexion Deformity If elbow can extend to a right angle  no treatment Occasionally necessary to treat a more marked flexion contracture by fractional lengthening of biceps & brachialis tendons  release of brachialis origin
  • 146. Forearm Pronation Deformity Fairly common  subluxation / dislocation of radial head Simple release of pronator teres or tendon can be rerouted round back of forearm  act as a supinator
  • 147. Wrist Flexion Deformity  Usually in an ulnar direction  improved by lengthening or releasing FCU  If extension is weak, released flexor tendon is transferred into one of wrist extensors  Severe cases  wrist arthrodesis with excision of proximal carpal row  cosmetic rather than functional benefit
  • 148. Flexion Deformity of The Fingers  Spasticity of long flexor muscles  clawing  Flexor tendons can be lengthened individually  Severe deformity  forearm muscle slide more appropriate  If fingers can be unclenched only by simultaneously flexing wrist, obviously important not to extend wrist by tendon transfer or fusion
  • 149. Thumb-in-palm deformity  Due to spasticity of thumb adductors or flexors (or both), but later there is also contracture of FPL  Mild cases  function can be improved by splinting thumb away from palm, or by operative release of adductor pollicis & 1st dorsal interosseus muscles  Resistant deformity  combined lengthening of FPL & release of thenar muscles, followed by tendon transfers  reinforce abduction & extension
  • 151. Regional Survey : Lower Limb SPASTIC HEMIPLEGIA Foot/ankle Tibialis anterior  invariably weak  equinovarus foot deformity Active plantar flexion to assist knee extension during stance phase  care when considering lengthening of gastroknemius / soleus complex Perform muscle recession rather than tendon lengthening
  • 152. Dynamic varus deformity Treated by a split tibialis anterior tendon transfer to outer side of foot  only ½ is transferred  to avoid risk of overcorrection into valgus Older children with fixed deformity  formal muscle lengthening with or w/o calcaneal osteotomy
  • 153. Pes Valgus  May require subtalar arthrodesis
  • 154. Hip/knee  Surgery is not usually required LLD  Discrepancies in growth  often short irrespective of any joint contracture  Epiphyseodesis of contralateral distal femoral and/or proximal tibial physes  can improve some aspects of gait pattern
  • 155. SPASTIC DIPLEGIA  Treatment is concentrated on lower limbs  Very young child  physiotherapy & splintage  prevent fixed contractures  Surgery  to correct structural defects (fixed contracture, hip subluxation), improve gait  3-4 yo  sitting & walking pattern  interrelationship between various postural defects, esp lumbar lordosis/hip flexion, knee flexion/ankle equinus
  • 156.  Most children will walk but delayed in learning to master this  Not walking by 6-7 yo is unlikely to do so  Non-ambulant children often have orthopedic problems similar to those with total body involvement  Walking diplegics  observational gait analysis is important & computerized gait analysis may have role in guiding treatment  each limb assessed independently
  • 157. Hip adduction deformity  Walks with thighs together, sometimes scissors gait  May be combined with spastic internal rotation  Adductor release is indicated if passive abduction <20 degrees on each side  Medial hamstring lengthening  done first  this alone may restore some hip abduction  Most patients open tenotomy of adductor longus & division of gracilis will suffice  if fails, other adductors be released  Anterior branch obturator neurectomy shouldn’t be performed
  • 158. Hip flexion deformity  Often associated with fixed knee flexion  walks with ‘sitting’ posture or hyperextension lumbar spine  Hip deformity >30o  operative  Walking child  not to weaken hip flexion too much  intramuscular lengthening of psoas tendon at pelvic brim is advocated  Non-walking child  psoas release at level of lesser trochanter is allowed  Associated fixed flexion deformity of knee may require medial hamstring lengthening
  • 159. Hip internal rotation deformity  Usually associated with flexion & adduction  adductor release & psoas lengthening will be helpful  After a few years rotation still excessive  derotation osteotomy of femur (subtrochanteric or supracondylar)  this may have to be followed by compensatory rotation osteotomy of tibia
  • 160. Hip subluxation  In 30% CP children  Persistent flexion-adduction deformity  femoral neck anteversion  Weak abductors & not fully weightbearing  risk of acetabular dysplasia & subluxation of joint  in non- walkers, may be complete dislocation  Correction of flexion & adduction deformities before 6 yo may have a role in preventing subluxation
  • 163. Older children may need varus-derotation osteotomy of femur, perhaps combined with acetabular reconstruction Longstanding dislocation in non-walker may be impossible to reconstruct; if discomfort makes operation imperative, proximal end of femur can be excised Adult walking displegic patient  total hip replacement in cases where painful degenerative change is affecting function
  • 164. Knee flexion deformity  One of commonest deformities  Usually due to functional hamstring tightness, often aggravated by hip flexion or weakness of ankle plantar flexion  Spastic flexion deformity may be revealed only when hip flexed to 90o  hamstrings tightened  Capsular contracture of knee joint is uncommon  Gait analysis  deciding hamstrings truly short or only functionally short
  • 165.  Fractional lengthening of hamstrings (medial more often)  improve gait mechanics  risks weakening hip extension & exacerbating hip flexion/lumbar lordosis  hamstrings normally assist with hip extension  Fractional lengthening of semimembranosus can be combined with detachment & transfer of semitendinosus to adductor tubercle at distal end of femur  Good results (Ma et al, 2006) in children with bilateral spastic flexion deformities >15 o combined with flexed- knee posture when standing or walking & ability to stand & walk only with support
  • 166. Severe flexion deformities (>25-30o)  extension osteotomy of distal femur or physeal plating anteriorly Knee extension is aided by plantarflexionof foot in walking  important not to weaken triceps surae by overzealous lengthening of Achilles tendon Spastic knee extension Simple tenotomyof proximal end of rectus femoris
  • 167. External tibial torsion Supramalleolar osteotomy First ensure that deformity is not actually advantageous in compensating for ankle/hindfoot deformity
  • 168. Equinus of the foot  Usually toe-walks  triggers excessive plantar-flexion-knee extension couple  manifested as knee hyperextension  Children with limited dorsiflexion  gastrocnemius is often more affected than soleus  Selective fractional lengthening of fascia/muscle is gaining favour but judicious percutaneous lengthening of Achilles tendon still popular  Relative overlengthening  problem, particularly when associated knee flexion contractures exist
  • 169.  If varus deformity is present, treatment is as for hemiplegic patient  More common deformity  equinovalgus and a ‘rocker- bottom’ foot  makes use of splints difficult & disrupts plantarflexion-knee extension couple, exacerbating knee flexion posture  Important to note whether hindfoot deformity is reducible or not  Calcaneal lengthening or displacement osteotomy but often subtalar fusion is required
  • 170. Such surgery must combined with release of tight structures (eg. Achilles tendon) & possibly peroneal lengthening & plication of medial structures when appropriate External tibial torsion  supramalleolar osteotomy but remember that externally rotated gait pattern may be compensating for an inability of foot to clear the ground when walking because of weak muscles / stiff joints
  • 171. Single event multi-level surgery (SEMLS)  Usually has problems at all levels  Enhance mechanical efficiency of gait by combaining changes at hip, knee & ankle  Soft tissue & bony surgery to both limbs can be performed at one sitting or staged over a few weeks  Postoperative rehabilitation is complex & time- consuming but results can be very rewarding
  • 173. Total Body Involvement Hip  Hip subluxation progressing to dislocation is common  Adduction & flexion contractures more frequent & severe  risk of developing subluxation with acetabular dysplasia  Often ‘windswept’  one hip lying adducted, flexed, internally rotated while other lies in abduction & external rotation & often more extended  release hip abductors & extensors  gluteus maximus & iliotibial band
  • 174.  Hip subluxation (>30% uncovering of femoral head) may require femoral varus derotation (& shortening) osteotomy as well as acetabular procedure for correction in addition to soft-tissue releases  Hip has dislocated  open reduction, release soft tissue & bony realignment  Alternative  proximal femoral resection  Complex surgery & high complication rates
  • 175. Spine / Pelvis  Scoliosis is very common (>50%)  Often a long C-shaped thoracolumbar curve  frequently incorporates pelvis which is tilted obliquely so that one hip is abducted & other adducted & threatening to dislocate  Trunk muscle involvement due to CP  major determinant of developing deformity  Various forms of non-operative treatment  some cases opt for long- term use of adapted wheelchair
  • 176. Indications for surgery :  Progressive curve >40o in a child >10 yo  Inability to sit w/o support  Range of hip movement that will allow child to sit after spinal stabilization  Fixation with pedicle screws & rods extending from thoracic spine to pelvis  Recreate lumbar lordosis  at least temporarily, exacerbate hamstring tightness making sitting more difficult
  • 177.  Complications :  Neurological defects  Problems with wound healing  Implant failure  This type of spinal surgery   life expectancy, but demonstrating concurrent improvement in quality of life has been more difficult to prove Other joints  Surgery may be required & follows principles outlined for hemiplegic & diplegic patient
  • 179. Cerebral damage following stroke or head injury  persistent spastic paresis in adult  can be accompanied by disturbance of propioception & stereognosis Early recuperative stage  physiotherapy & splintage  prevent fixed deformities  all affected joints should be put through full range of movement every day Botulinum toxin may be beneficial in resistant cases
  • 180.  Deformities that passively correctible should be splinted in neutral position until controlled muscle power returns  Propioception & coordination  occupational th/  Once max motor recovery has been achieved (9 mo after stroke but >1 year after brain injury), residual deformities or joint instability should be considered for operative  Sufficient cognitive ability, awareness of body position in space, good phychological impetus  if lasting result is to be expected
  • 181. Lower limbs  principal deformities requiring correction  equinus or equinovarus of foot, flexion of knee, adduction of hip Upper limb  chances regaining controlled movement <)  common residual deformities  adduction & internal rotation of shoulder (often by shoulder pain), flexion of elbow, wrist, MCP joints
  • 183.  Though rare  most common of hereditary ataxias  Autosomal recessive condition  detected on genetic testing  a triplet expansion  localized to chromosome 9  In USA, about 1 in 90 adults is a carrier  In childhood (rarely adulthood) & all patients develop progressive ataxia of limbs & of their gait with associated extensor plantar responses but absent knee & ankle reflexes & sensory disturbances (loss of vibration sense & 2-point discrimination)  Dysarthria appears within 5 years of onset
  • 184. Neurological degeneration in spinocerebellar tracts, corticospinal tracts, posterior columns of spinal cord & parts of cerebellum Slowed motor velocities in median & tibial nerves with absent sensory action potentials in sural & digital nerves Painful muscle spasms occur in some patients  tend to worsen with time
  • 185.  More common  progressive cavo-varus foot deformity  rigid, clawed toes & scoliosis  The earlier onset of disease the greater is risk of significant curve progression  In more severe cases, functional & neurological deterioration may be rapid with cardiomyopathy & death in early to mid adulthood  In other more mild cases, surgical correction of foot & spine deformities may be worthwhile
  • 186. Lesions of The Spinal Cord
  • 187. 3 major pathways in spinal cord :  corticospinal tracts (in the anterior columns)  motor neurons  spinothalamic tracts  sensory neurons  pain, touch, temperature  posterior column tracts  deep sensibility  joint position, vibration
  • 189. Clinical features  True lesions  UMN spastic paresis & often a fairly precise sensory level  Extradural compressive lesions will often involve nerve roots  combination of UMN & LMN signs  Weakness & numbness with loss of balance & possibly alteration in bowel or bladder control, impotence  Several 'classical' patterns are recognized
  • 190. Cervical cord compression UMN symptoms in lower limbs (stiffness & change in gait pattern) & LMN signs in upper limbs (numbness & clumsiness) Pain  variable feature Bladder symptoms are of frequency & incontinence more commonly than retention
  • 192. A central cord syndrome Hyperextension injury in middle-aged patient with longstanding cervical spondylosis, or may develop in syringomyelia Disproportionately more UMN weakness in upper limbs compared to lower limbs  bladder dysfunction & a variable sensory loss below lesion
  • 193.  Thoracic cord compression UMN paralysis affecting lower limbs  variable sensory loss depending on degree of involvement of dorsal columns or spinothalamic tracts  Lumbar cord compression Spinal cord terminates around L1  conus medullaris or cauda equina or both  mixture of UMN & LMN signs  Typical cauda equina syndrome  lower limb weakness, absent reflexes, impaired sensation & urinary retention
  • 194. Brown-Sequard lesion  Pure form  very unusual  incomplete hemispherical cord lesion :  Below lesion  ipsilateral UMN weakness & posterior column dysfunction, contralateral loss of skin sensibility  At level of lesion  ipsilateral loss of sensibility  Less pure forms  common
  • 197. Spinal shock Acute cord lesions at any level  flaccid paralysis which resolves over time, usually to reveal more typical UMN signs associated with cord injury
  • 198. Causes of spinal cord dysfunction
  • 200. Diagnosis & management Traumatic & compressive lesions  most likely to be seen Plain x-rays  structural abnormalities of spine; cord compressioncan  myelography alone / combined with CT Intrinsic lesions of cord  blood tests, CSF examination & MRI
  • 201. Acute compressive lesions  urgent diagnosis & treatment if permanent damage is to be prevented Bladder dysfunction is ominous  motor & sensory signs may improve after decompression Loss of bladder control >24 hours  irreversible
  • 202. Any spinal injury may be associated with cord damage  great care in transporting & examining patient In early period of 'spinal shock'  flaccid paralysis, with or without priapism Plain x-rays seldom show full extent of bone displacement  much better by CT / MRI
  • 203. Unstable injuries  operative decompression and/or stabilization; stable injuries  conservatively Many centres consider use of corticosteroids  reducing degree of permanent neurological damage  side effects : GI haemorrhage & arrascular necrosis
  • 204.  Epidural abscess  surgical emergency  acute pain & muscle spasm, fever, leucocytosis,  ESR  X-rays  disc space narrowing & bone erosion  immediate decompression & antibiotics  Acute disc prolapse  unilateral symptoms & signs  Complete lumbar disc prolapse  cauda equina syndrome  urinary retention & overflow  Spinal canal obstruction  MRI  Operative discectomy  urgent
  • 205.  Chronic discogenic disease  narrowing of intervertebral foramina & compression of nerve roots (radiculopathy), bone hypertrophy, pressure on spinal cord (myelopathy)  x-ray & MRI  operative decompression  Spinal stenosis  direct pressure on cord / nerve roots, vascular obstruction, ischaemic neuropathy during hyperextension of lumbar spine  'tiredness', weakness, aching / paraesthesia in lower limbs after standing / walking for a few minutes  relieved by bending forward, sitting / crouching so as to flex lumbar spine
  • 206.  Congenital narrowing of spinal canal  rare, except in developmental disorders (achondroplasia)  bony decompression of nerve structures  Vertebal disease  TB / metastatic disease  cord compression & paraparesis  x-ray  needle biopsy for confirmation  anterior decompression & internal stabilization, radiotherapy, corticosteroids, narcotics
  • 207.  Spinal cord tumours  comparatively rare  progressive paraparesis  X-rays  bony erosion, widening of spinal canal / flattening of vertebral pedicles  Widening of intervertebral foramina  typical of neurofibromatosis  operative removal of the tumour  Intrinsic lesions of cord  slowly progressive neurological signs  tabes dorsalis & syringomyelia  neuropathic joint destruction
  • 208.  Tabes dorsalis  late manifestation of syphilis  degeneration of posterior columns of spinal cord  'lightning pains' in lower limbs  Sensory ataxia  stamping gait; loss of position sense & pain sensibility; trophic lesions in lower limbs; progressive joint instability; almost painless destruction of joints (Charcot joints)  No treatment for cord disorder
  • 209.  Syringomyelia  long cavity (the syrinx) filled with CSF develops within spinal cord  usually the cause is unknown, sometimes associated with tumours / SCI & congenital anomalies (hidrocephalus & herniation of cerebellar tonsils)  Symptoms & signs  most noticeable in upper limbs  expanding cyst presses on anterior horn cells  weakness & wasting of hand muscles
  • 210.  Destruction of decussating spinothalamic fibres in centre of cord  sensory loss in upper limbs: impaired response to pain & temperature but preservation of touch  Trophic lesions in fingers & neuropathic arthropathy ('Charcot joints') in upper limbs  CT  expanded cord & syrinx  MRI  Deterioration may be slowed down by decompression of foramen magnum
  • 212.  Congenital disorder  2 halves of posterior vertebral arch fail to fuse at ≥1 levels  Neural tube defect, or spiral dysraphism  1st month of foetal life  lumbar / lumbosacral  Most severe form  major neurological problems in lower limbs & incontinence SPINA BIFIDA
  • 213. Spina bifida occulta  Mildest forms of dysraphism  midline defect between the laminae & nothing more  Usually L5  Telltale defects in overlying skin  dimple, pit, tuft of hair  Tethering of conus medullaris below L1, splitting of spinal cord (diastematomyelia), cysts / lipomas of cauda equina
  • 214. Spina bifida cystica  Vertebral laminae  missing & contents of vertebral canal prolapse through defect  Abnormality takes 1 of several forms  Least disabling  meningocele  5%  Duramater open posteriorly, meninges intact, CSF-filled meningeal sac protrudes under skin  Spinal cord & nerve roots remain inside vertebral canal  no neurological abnormality
  • 215. Myelomeningocele  Most common & serious abnormality  lower thoracic spine / lumbosacral  Part of spinal cord & nerve roots prolapse into meningeal sac  Neural tube fully formed & sac covered by membrane and/or skin  closed myelomeningocele  In others cord in unfolded neural plate forming roof of sac  open myelomeningocele  neurological deficit distal to level of lesion  If neural tissue exposed to air  infected  more severe abnormality & death
  • 216. Hydrocephalus Distal tethering of cord  herniation of cerebellum & brain-stem through foramen magnum  obstruction to CSF circulation & hydrocephalus Ventricles dilate & skull enlarges by separation of cranial sutures Persistently raised intracranial pressure  cerebral atrophy & learning difficulties
  • 217. lncidence & screening Isolated laminar defects  >5% of lumbar spine x-rays Cystic spina bifida  rare  2-3/1000 live births If 1 child is affected  risk for future siblings significantly 
  • 219. Neural tube defects  high levels of AFP in amniotic fluid & serum  ANC 15-18th week of pregnancy. Maternal blood testing  15-18 weeks & followed by an amniocentesis if necessary Mid-term high resolution USG  95% Folic acid 400 micrograms/day  continuing through the first 12 weeks of pregnancy
  • 222. ClinicaI features EARLY DIAGNOSIS  Spina bifida occulta  enuresis, urinary frequency / intermittent incontinence; weakness, some loss of sensibility in lower limbs  Plain x-rays  laminar defect & any associated vertebral anomalies; midline ridge of bone  bifurcation of cord (diastematomyelia)  Intraspinal anomalies  MRI
  • 223. Spina bifida cystica  Saccular lesion overlying lumbar spine  Open myelomeningoceles  plum coloured skin  1/3 infants  complete LMN paralysis & loss of sensation & sphincter control below affected level  X-rays & CT  extent of bony lesion + other vertebral anomalies  MRI  define neurological defects
  • 224. Clinical features in older children Clawing toes, change in gait pattern, incontinence / abnormal sensation  tethered cord syndrome MRI with gadolinium Neurosurgical release Liable to suffer fractures after minor injuries
  • 225. Treatment  Intrauterine surgery  closure of defect  Formal neurological closure  <48 hours of birth  prevent drying, ulceration, infection  Some centres avoid urgent operation if above L1 / very severe spinal deformities / marked hydrocephalus  90% need VP shunt   risk of further damage to CNS
  • 226. Neurological status changes unexpectedly  shunt infection / blockage Physiotherapy and/or splinting  mainstays of early treatment Vast majority of patients  urological problems  catheters / urinary diversion  botulinum toxin injections   capacity & improve continence
  • 228. ORTHOPAEDIC MANAGEMENT  Except in mildest cases, late functional outcome cannot be predicted until child is assessed intellectually & neuromuscular unction  3-4 years  For many patients, ability to sit comfortably is more important than to stand awkwardly  Best predictor of walking ability & function  motor level of paralysis
  • 229. Lesions <L4  quadriceps control & active knee extension Higher lesions  wheelchair Immobilization & muscle imbalance  joint deformity & risk ofpathological # Latex allergy is present in some children with spina bifida Th/: antihistamines and/or corticosteroids
  • 230. Spine  Scoliosis and/or kyphosis is common in children with myelomeningocele  muscle weakness & imbalance, congenital vertebral anomalies (20 % cases) & tethered cord syndrome  Distal tethering of cord / other neural elements  almost inevitable after repair of myelomeningocele  harmless  pain & progression of neurological dysfunction  PPS
  • 231. Kyphosis  Stretching & breakdown / chronic ulceration of overlying skin posteriorly & compression of abdominal & thoracic viscera anteriorly  Treatment  difficult  localized vertebral resection & arthrodesis  Cord at affected level ofter non-functioning  risks of further neurological insult influencirrg outcome are small
  • 232. Paralytic scoliosis  Long C-shaped curve  progressive  sitting difficult  Unlikely to respond to brace  Molded seat inserts for wheelchair  help  rate of curve deterioration  Surgery via anterior, posterior or combined apprroach; fusion to pelvis
  • 233. Hip  General aim  to secure hips  enable child to stand in calipers & to sit comfortably  Neurological lesion >L1  all muscle groups are flaccid  splintage & wheelchair  Lesions <S1  hip flexion contracture  elongation of psoas tendon + detachment of flexors from ilium  Soutter operation
  • 236. Muscle imbalance  bilateral hip dislocation
  • 237. ‘In between’ lesions  muscle imbalance  hip sublux / dislocate Retaining hip movement may be more useful than striving for hip reduction by multiple operations
  • 238. Knee Aim  straight knee for callipers & using gait- training devices Older children  prolonged sitting  fixed flexion  stretching (by distraction) fails  ≥1 hamstrings lengthened, divided / reinserted into femur / patella; posterior capsular release
  • 239. Hyperextension contracture & hamstring tendons subluxed anteriorly  physiotherapy & serial casting  V-Y quadricepsplasty & hamstring lengthening Walking patients  valgus knee, torsional abnormalities in lower limb Secondary joint instability  further exacerbate problems of walking  forearm crutches & swing-through gait
  • 240. Foot Most common problem Aim  mobile foot, healthy skin & soft tissues  can be held / braced in plantigrade position Flail foot / balanced paralysis / weakness  accurately made orthoses (eg. ankle-foot orthosis), well-fitting ankle boots Equinovarus deformity  standard treatment : aggressive soft-tissue release
  • 241. Bony procedures  for residual / recurrent deformity in older child Vertical talus deformity  ‘reverse Ponseti’ & transfer of tibialis anterior tendon to neck talus Toe deformities  ‘orthopaedic shoes’ with high toe box
  • 243.  Acute infectious viral disease  Spread by oropharyngeal route  10% exhibit any symptoms at all & 1% effects on anterior horn cells of spinal cord & brain stem  LMN paralysis  Acute illness resembling meningitis to paralysis, slow recovery / convalescence, long period of residual paralysis  At any age but most commonly in children
  • 244. Acute illness  fever, headache  1/3 patients  sore throat, mild headache, slight pyrexia 5-7 days before  neck stiffness  Patient curled up  passive stretching provoke painful spasms Paralysis  peak at 2-3 days  breathing & swallowing difficulty  Pain & pyrexia subside after 7-10 days  Infective for 4 weeks from onset
  • 245. Recovery & convalescence  first 6 months up to 2 years Residual paralysis  some degree of asymmetric flaccid (LMN) paralysis / unbalanced muscle weakness  joint deformities & growth defects Sensation intact, but limb often cold & blue
  • 246. Post-polio syndrome  up to 50% reactivation of virus  progressive muscle weakness in old & new muscle groups  unaccustomed fatigue Confirmed poliomyelitis + neurological stability min 15 years The older the child at onset, more severe the disease & more likely the adult would develop PPS
  • 252. Early treatment Acute phase  isolated, symptomatic treatment for pain & muscle spasm, gentle passive stretching, artificial respiration Acute illness settles  physiotherapy Between exercise periods, splintage Muscle charting at regular intervals until no further recovery detected
  • 253. Late treatment  Isolated muscle weakness without deformity  instability (quadriceps paralysis) / loss of complex function (thumb opposition  tendon transfer)  Passively correctible deformity  appropriate tendon transfer may solve problem permanently Muscle usually loses 1 grade power when transferred Grade 3 muscle  tenodesis  reduce deformity by gravity
  • 255. Fixed deformity  operatively stabilize joint  arthrodesis  ankle, foot, paralytic scoliosis Occasionally beneficial  equinus foot to compensate mechanically for quadriceps weakness Flail joint  if unstable, permanent splintage / arthrodesis
  • 257. Shortening  Normal bone growth depends on normal muscle activity  children with poliomyelitis in early years can develop difference in leg length  LLD 3-5 cm  shoe raise  LLD can be mitigated by well-timed epiphyseodesis in normal limb
  • 258. Disturbance of skeletal modelling  torsional / angular deformities in sagittal / coronal plane Muscle & joint contractures may aggravate effects of bone distortion Vascular dysfunction  large chilblains  sympathectomy
  • 259. Shoulder Strong scapular muscles  abduction at shoulder can be restored by arthrodesing gleno-humeral joint (50o abducted & 25o flexed) Contracted adductors may need division
  • 260. Elbow & forearm  Normal power in anterior forearm muscles  common flexor origin moved more proximally on distal humerus  Strong pectoralis major  lower half of muscle detached & join biceps tendon  Pronation of forearm  transposing active FCU tendon across front of forearm to radial border  Supination loss  transposing FCU across back of forearm to distal radius
  • 261. Wrist & hand  Deformity / instability  arthrodesis  Any active muscles can be used to restore finger movement  Opposition weakness  flexor superficialis transfer  tendon wound round FCU, threaded across palm & fixed to distal end of 1st MC
  • 262. Lister GD: The Hand: Diagnosis and Indications, ed 2. Edinburgh, Scotland: Churchill
  • 263. Trunk Unbalanced paralysis  scoliosis  long thoracolumbar curve  lumbosacral junction  pelvic obliquity Most effective operation : anterior & posterior instrumentation & fusion
  • 264. Hip  Paralysis usually <5 yo  persistent anteversion of femoral neck, coxa valga, underdevelopment of acetabular socket  Keys : reduce any scoliotic pelvic obliquity, overcome muscle imbalance, correct prox femoral deformity by intertrochanteric / subtrochanteric osteotomy, acetabuloplasty to deepen acetabular socket  Fixed abduction with pelvic obliquity  fascia lata & ITB may need division
  • 265. Knee Instability  relative weakness of knee extensors Good extensor power hip + good foot plantarflexion power (or fixed equinus) + knee thrust into hyperextension  unaided walking Weak hip / ankle joints  full-length calliper / supracondylar extension osteotomy of femur
  • 266. Fixed flexion with flexors stronger than extensors is more common Flexor-to-extensor transfer  hamstring muscles to patella / quadriceps tendon If flexors not strong enough  supracondylar extension osteotomy Genu recurvatum  supracondylar extension osteotomy / excise patella & slot into upper tibia
  • 270. Foot  Foot drop  ankle-foot orthosis / below-knee calliper  Varus, valgus, calcaneocavus  fusion + tendon re- routing  Varus, valgus  Grice, Dunn  Foot drop  Lambrinudi  Calcaneocavus  Elmslie
  • 271. Grice
  • 275. Dunn A, Position of skin incision (broken line) and amount of bone resected (colored area). B, Position of bones after surgery. Foot (except for talus) has been displaced posteriorly at subtalar joint so that
  • 281. Claw toes If mobile  transfer toe flexors to extensors If fixed  IP joint should be arthrodesed in straight position & long extensor tendons reinserted into metatarsal necks
  • 283. Motor neuron disease (amyotrophic lateral sclerosis)  Degenerative disease, unknown etiology  UMN & LMN symptoms & signs  Middle age patient  Dysarthria, difficulty in swallowing, muscle weakness, wasting, exaggerated reflexes  Muscle cramps, atrophy, fasciculation
  • 284. Progressive, incurable Some have frontotemporal dementia / pseudobulbar effect  emotional lability Most die within 5 years  respiratory weakness + aspiration pneumonia
  • 285. Spinal Muscular Atrophy  Heritable (defect on long arm of chromosome 5)  Widespread degeneration of anterior horn cells in cord  progressive LMN weakness  Commonest : Werdnig-Hoffman disease  autosomal recessive  floppy & weak baby, difficult feeding & breathing  death in 1 year  Less severe : Kugelberg-Welander disease  dominant / recessive  adolescents / young adult
  • 289. Classification  By anatomical level & distribution  >40% cases  no specific cause is found  Radiculopathy  nerve roots  trauma, intervertebral disc herniation / bony spur, SOL of spinal canal, root infection  Plexopathy  direct trauma, compression by local tumours, entrapment in thoracic outlet syndrome, viral infection
  • 291. Entrapment in thoracic outlet syndrome
  • 292. Neuralgic amyotrophy A. On the left: atrophy of supraspinatus and infraspinatus muscles and rhomboid muscles (white arrow); on the right: scapular tilting and rotation caused by serratus anterior muscle weakness (white arrow with *) B. On the right: severe scapular winging caused by serratus anterior paralysis
  • 293. C. On the left: atrophy of supraspinatus and infraspinatus muscles (white arrow), and trapezius muscle (white arrow with *) showing underlying rhomboid muscles D. Severe atrophy of the deltoid muscle (white arrow) and moderate atrophy of the biceps brachii muscle (white arrow with *)
  • 294. Distal neuronopathy : Mononeuropathy  single nerve, mixed sensorimotor Multiple mononeuropathy  several isolated nerves  leprosy, diabetes, vasculitis Polyneuropathy  widespread symmetrical dysfunction
  • 295. Pathology  Large nerve fibres   >4 µm  myelinated  Small fibres   <4 µm  unmyelinated  Acute interruption of axonal continuity  Axonal degeneration  Demyelination
  • 296. Acute Axonal Interruption After nerve division Loss of motor & sensory  immediate & complete Muscle fibres will degenerate if nerve conduction not restored in 2 years NCV & EMG Axon grows ±1 mm / day, often incomplete
  • 297. Chronic Axonal Degeneration  Non-traumatic  Large fibres  ‘stocking’ & ‘glove’ numbness, altered postural reflexes, ataxia, muscle weakness & wasting  Feet & legs before hands & arms  NVC  CMAP & SNAP  & EMG  Small fibres  orthostatic hypotension, cardiac arrhythmias,  peripheral limb perfusion, ischaemia, limb infection, burning dysaesthesias
  • 298. Demyelinating Neuropathies  Nerve entrapment syndrome & blunt soft- tissue trauma  Sensory and/or motor dysfunction distal to lesion  Recovery <6 weeks  Rare, exeption: Guillain- Barre syndrome
  • 299. Clinical features ‘pins & needles’ numbness, limb ‘going to sleep’, ‘burning’, shooting pains, restless legs Weakness, clumsiness, loss of balance in walking Pain sensibility & propioception depressed  Charcot joints
  • 302. Hereditary sensory neuropathy Dominant or recessive trait Neuropathic joint disease & ulceration of feet Cycle of painless injury & progressive deformity  severe disability
  • 304. Hereditary motor & sensory neuropathy (HMSN) Peroneal muscular atrophy, Charcot-Maria-Tooth disease, benign forms of spinal muscular atrophy Autosomal dominant HMSN type I  children  difficulty walking, claw toes, pes cavus / cavovarus  confirmed by demyelination on sural nerve biopsy / genetic test of blood samples
  • 305. "Stork legs"  most noticeable distal to knees
  • 307.  HMSN type II  adolescents & young adults  much less disabling than type I  affect only lower limbs  mild pes cavus & wasting of peronei  Early stages  foot & ankle orthoses  Claw toes  transfer toe flexors to extensors, with / wo fusion of IP joints  Clawing of big toe  Robert Jones procedure  transfer EHL to metatarsal neck & fusion of IP joint  Cavus deformity  calcaneal / dorsal mid-tarsal osteotomy / triple arthrodesis
  • 309. Familial liability to pressure palsy (HNPP) Dominant disorder Multiple mixed entrapment mononeuropathies  CTS + ulnar nerve palsy
  • 310. Friedreich’s Ataxia  Autosomal recessive  Spinocerebellar ataxia: spinocerebellar dysfunction and may be also be degeneration of the posterior root ganglia and peripheral nerve.  Present at 6 y.o, with ataxic gait, lower limb weakness.  The muscle weakness is progressive -> at 20 y.o. use wheelchair -> die due to cardiomyopathy before 45 y.o
  • 313. Diabetic Neuropathy  Asscociated with hyperglycemia, interfere axon and schwann cell function -> demyelination and axonal degeneration  The onset is insidious  Condition often goes undiagnosed until patients start complaining of numbness and paraesthesiae in the feet and lower legs  Complications: neuropathic ulcers of the feet, regional osteoporosis, insufficiency fractures of the foot bones, or Charcot joints in the ankles and feet Treatment:  skin care, management of fractures and splintage or arthrodesis of grossly unstable or deformed joints
  • 314. Alcoholic neuropathy  the main cause is the accompanying nutritional deficiency, especially thiamine deficiency  Symptoms: ‘burning’, paraesthesiae, numbness and muscle weakness in the feet and legs  Treatment: nutritional supplementation, administration of thiamine, protection from trauma
  • 315. INFECTIVE NEUROPATHY  caused by the varicella  dormant for many years in the dorsal root ganglia, is then reactivated and migrates down the nerve  Symptoms:  the patient develops severe unilateral pain in the distribution of several adjacent nerve roots  Days or weeks later an irritating vesicular rash appears; characteristically it trails out along the dermatomes corresponding to affected nerves Herpes Zoster (shingles) This patient was treated for several weeks for ‘sciatica’ – then the typical rash of shingles appeared
  • 316. Neuralgic amyotrophy (acute brachial neuritis)  unusual cause of severe shoulder girdle pain and weakness is believed to be due to a para- infectious disorder of one or more of the cervical nerve roots and the brachial plexus  Pain in the shoulder and arm is typically sudden in onset,intense and unabating  Other symptoms are paraesthesiae in the arm or hand and weakness of the muscles of the shoulder, forearm and hand
  • 317.  Winging of the scapula (due to serratus anterior weakness), wasting of the shoulder girdle muscles, and occasionally involvement of more distal arm muscles may be profound, becoming evident as the pain improves  Sensory loss and paraesthesiae in one or more of the cervical dermatomes is common  no specific treatment; pain is controlled with analgesics
  • 318. Guillain–Barré syndrome (acute inflammatory demyelinating polyneuropathy – AIDP)  acute demyelinating motor and sensory (though mainly motor) polyneuropathy  can occur at any age and usually appears two or three weeks after an upper respiratory or gastrointestinal infection – probably as an autoimmune reaction
  • 319.  Cerebrospinal fluid analysis may show a characteristic pattern: elevated protein concentration in the presence of a normal cell count  Nerve conduction studies may show conduction slowing or block; in severe cases there may be EMG signs of axonal damage  Treatment consists essentially of bed rest, pain-relieving medication and supportive management to monitor, prevent and deal with complications such as respiratory failure and difficulty with swallowing
  • 320. Leprosy  Mycobacterium leprae, causes a diffuse inflammatory disorder of the skin, mucous membranes and peripheral nerves  In tuberculoid leprosy, anaesthetic skin patches develop over the extensor surfaces of the limbs; loss of motor function leads to weakness and deformities of the hands and feet  Treatment by combined chemotherapy (mainly rifampicin and dapsone) is continued for 6 months to 2 years
  • 321. PAIN Pain receptors  stimulated by mechanical distortion, by chemical, thermal or electrical irritation, or by ischaemia  Musculoskeletal pain associated with trauma or inflammation is due to both tissue distortion and chemical irritation (local release of kinins, prostaglandins and serotonin)  Visceral nociceptors respond to stretching and anoxia Pain perception
  • 322. Pain transmission  transmitted via both myelinated axons  From the dorsal horn synapses in the cord, some fibres participate in ipsilateral reflex motor and autonomic activities while others connect with axons in the contralateral spinothalamic tracts that run to the thalamus and cortex
  • 323. Pain modulation  Pain impulses may be suppressed or inhibited by  simultaneous sensory impulses travelling via adjacent axons  impulses descending from the brain  pain impulses are ‘sorted out’ – some of them blocked, some allowed through – in the dorsal horn of the cord  certain morphine-like compounds (endorphins and enkephalins), normally elaborated in the brain and spinal cord, can inhibit pain sensibility Pain threshold  No fixed threshold for any individual  threshold is lowered by fear, anxiety, depression, lack of self-esteem and mental or physical fatigue  elevated by relaxation, diversion, reduction of anxiety and general psychological support
  • 324. Acute pain  Severe acute pain, is accompanied by an autonomic ‘fight or flight’ reaction:  Increased pulse rate  peripheral vasoconstriction  Sweating  Rapid breathing  muscle tension  Anxiety  Treatment is directed at: (1) removing or counteracting the painful disorder (2) splinting the painful area (3) making the patient feel comfortable and secure (4) administering analgesics, anti- inflammatory drugs or – if necessary – narcotic preparations (5) alleviating anxiety
  • 325. Chronic pain  occurs in degenerative and arthritic disorders or in malignant disease and is accompanied by vegetative features such as fatigue and depression  Treatment again involves alleviation of the underlying disorder if possible and general analgesic therapy  Need rehabilitation and psychologically support
  • 326. Complex regional pain syndrome (CRPS)  pain out of proportion (in both intensity and duration) to the precipitating cause,vasomotor instability, trophic skin changes, regional osteoporosis and functional impairment  Causes are trauma (often trivial), operation or arthroscopy, a peripheral nerve lesion, myocardial infarction, stroke and hemiplegia
  • 328. PATHOGENESIS  sympathetic overactivity  Abnormal cytokine release, neurogenic inflammation, sympathetic-mediated enhancement of pain responses CLINICAL FEATURES  Complains of burning pain, and sometimes cold intolerance, in the affected area – usually the hand or foot, sometimes the knee or hip, and sometimes the shoulder in hemiplegia  Local redness and warmth, sometimes changing to cyanosis with a blotchy, cold and sweaty skin  X-rays are at first usually normal
  • 329.  Causalgia is a severe form of regional pain, usually seen after a nerve injury  Pain is intense, often ‘burning’ or ‘penetrating’ and exacerbated by touching, jarring or sometimes even by a loud noise
  • 330. TREATMENT  Mild cases often respond to a simple regimen of reassurance, anti-inflammatory drugs and physiotherapy  Administration of corticosteroids, calcium channel blockers and tricyclic antidepressants  If there is no improvement after a few weeks sympathetic blockade often helps  Psychological treatment may help them to deal with the emotional distress and anxiety and to develop better coping strategies
  • 331. Chronic pain syndrome  well-marked features of depression, or complaints of widespread somatic illness (pain in various parts of the body, muscular weakness, paraesthesiae, palpitations and impotence)  Treatment is always difficult and should, ideally, be managed by a team that includes a specialist in pain control, a psychotherapist, a rehabilitation specialist and a social worker  Pain may be alleviated by a variety of measures: (1) analgesics and anti-inflammatory drugs; (2) local injections to painful areas; (3) local counter-irritants; (4) acupuncture; (5) transcutaneous nerve stimulation; (6) sympathetic block; (7) surgical interruption of pain pathways
  • 332. FIBROMYALGIA  complain of pain and tenderness in the muscles and other soft tissues around the back of the neck and shoulders and across the lower part of the back and the upper parts of the buttocks
  • 334. Muscular Dystrophies  Duchenne’s muscular dystrophy  Limb girdle dystrophies  Facioscapulohumeral dystrophy
  • 335. Duchenne’s muscular dystrophy  Inheritance with recessive transmission  seen only in boys (or in girls with sex chromosome disorders)
  • 336.  Clinical Appereance  difficulty standing and climbing stairs, he cannot run properly and he falls frequently  Weakness begins in the proximal muscles of the lower limbs and progresses distally, affecting particularly the glutei, the quadriceps and the tibialis anterior, giving rise to a wide-based stance and gait with the feet in equinus, the pelvis tilted forwards, the back arched in lordosis and the neck extended  Characteristic feature is the child’s method of rising from the floor by climbing up his own legs (Gowers’ sign); this is due to weakness of the gluteus maximus and thigh muscles  Cardiopulmonary failure is the usual cause of death, generally before the age of 30 years  Confirmation is achieved by muscle biopsy and genetic testing with a DNA polymerase chain reaction.
  • 337. Treatment  While the child can still walk -> physiotherapy and splintage  Corticosteroids are useful in preserving muscle strength  If scoliosis is marked (more than 30 degrees), instrumentation and spinal fusion helps to maintain pulmonary function and improves quality of life although not necessarily lifespan
  • 339. BECKER MUSCULAR DYSTROPHY  X-linked recessive disease, is similar to but milder than Duchenne’s dystrophy  Dystrophin is decreased and/or abnormal in character  The muscles of facial expression are not affected and neither are the muscles controlling bowel or bladder function or swallowing
  • 340. LIMB GIRDLE DYSTROPHY  characterized by :  weakness of the pelvic and shoulder girdle muscles  usually start in late adolescence  causes a waddling gait and difficulty in rising from a low chair  pectoral girdle weakness makes it difficult to raise the arms above the head Treatment  Physiotherapy  Splintage to prevent contractures,  operative correction when necessary
  • 341. FACIOSCAPULOHUMERAL DYSTROPHY  autosomal dominant condition with very variable expression  muscle weakness is first seen in  the face (inability to purse the lips or close the eyes tightly)  followed by weakness of scapular muscles causing winging of the scapula and difficulty with shoulder abduction  condition is due to gene deletion on the long arm of chromosome 4
  • 342. MYOTONIA  persistent muscle contraction after cessation of voluntary effort  Two type: 1. DYSTROPHIA MYOTONICA 2. MYOTONIA CONGENITA
  • 343. DYSTROPHIA MYOTONICA  autosomal dominant disorder with an incidence of about 1 in 7000  muscle stiffness for some years  systemic features appear – diabetes, cataracts and cardiorespiratory problems – and by middle age patients are often severely disabled  Treatment is essentially palliative but foot deformities may need manipulation and splintage
  • 344. MYOTONIA CONGENITA  inherited by autosomal recessive transmission  appear in childhood and usually progress slowly  typically this is worse after periods of inactivity and is relieved by exercise  triggered by exposure to cold and can cause pain (‘muscle cramps’)  There is no specific treatment for this condition.  Patients are advised about avoiding aggravating activities.

Hinweis der Redaktion

  1. 6 months twins developed quite differently Lack of head and arm control
  2. Lack of body control when helped to the sitting position
  3. Inability to sit unaided
  4. Hemiplegia  commonest  spastic palsy on 1 side of body with upper limb more severely affected than lower  most can walk & respond reasonably well to treatment Diplegia  both sides of body with lower limbs always most severely affected Side to side involvement may be asymmetrical  asymmetric diplegia, bilateral hemiplegia Many cases are secondary to prematurity & periventricular leucomalacia on brain MRI Intelligence is often normal Less severely  reasonable mobility
  5. Hemiplegic Diplegic Whole body
  6. Adductor spasm (scissor stance) Flexion deformity of hips and knees with equinus of the feet General posture and characteristic facial expression Ataxic type of palsy