This document provides information on neuromuscular disorders and the nervous system. It discusses the basic structure and function of neurons, nerves, and muscles. It describes the somatic motor and sensory systems, including reflex activity. It also covers the autonomic nervous system and peripheral nerves. Key aspects of skeletal muscle such as fiber types, contraction, and wasting are summarized. The document concludes with sections on clinical assessment, including history, examination techniques, imaging, and neurophysiological studies.
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
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
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
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
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
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
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
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
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
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
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
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
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 *)
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
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
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
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
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
6 months twins developed quite differently
Lack of head and arm control
Lack of body control when helped to the sitting position
Inability to sit unaided
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
Hemiplegic
Diplegic
Whole body
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