2. Upper motor neuron
Characteristic of upper motor neurone lesions:
no wasting;
increased tone of clasp-knife type;
weakness most evident in anti-gravity
muscles;
increased reflexes and clonus;
extensor plantar responses.
3. Upper motor neuron
Lesion situated peripherally in the cerebral
hemisphere produces weakness of part of
the contralateral side of the body
Lesion of the internal capsule, are much
more likely to produce weakness of the whole
of the contralateral side of the body & visual
loss
Lesion in the spinal cord in high cervical level
will cause ipsilateral hemiparessis, below
the neck level ipsilateral monoparessis
4. Lower motor neuron
characteristics of lower motor neurone lesions:
wasting;
fasciculation
decreased tone (i.e. flaccidity)
weakness
decreased or absent reflexes
flexor or absent plantar responses
Anterior horn
7. Amyotrophic Lateral Sclerosis
Degenerative disorder of upper and lower
motor neurons of the corticospinal tract
Anterior motor horn degeneration leads to
lower motor neuron signs
Lateral corticospinal tract degeneration leads
to upper motor neuron signs
8. Amyotrophic Lateral Sclerosis
The process is remarkably selective, leaving
special senses, and cerebellar, sensory and
autonomic functions intact
Tends to start either as a problem in the bulbar
muscles, or as a problem in the limbs
Initially the involvement tends to be either
lower motor neurone or upper motor neurone
in nature
the coexistence of both, in the absence of
sensory signs, is the hallmark of motor
neurone disease
9. pathogenesis
Most cases are sporadic, arising in middle age
adults.
Zinc-copper superoxide dismutase mutation
(SOD1) is present in some familial cases;
leads to free radical injury in neurons
Prior to their destruction, motor neurons
develop protein-rich inclusions in their cell
bodies and axons containing ubiquitin
10. Clinical presentation
In 75-80% of patients, symptoms begin with limb
involvement.
Initial complaints in patients with lower limb onset are often
as follows:
Tripping, stumbling, or awkwardness when running
Foot drop; patients may report a "slapping" gait
Initial complaints with upper limb onset include the
following:
Reduced finger dexterity, cramping, stiffness, and
weakness or wasting of intrinsic hand muscles
Wrist drop interfering with work performance
With bulbar onset (20-25%), initial complaints are as
follows:
Slurred speech, hoarseness, or decreased volume of
speech
11. Clinical presentation
Emotional and special cognitive difficulties in some
ALS patients are as follows:
Involuntary laughing or crying
Depression
Impaired executive function
Maladaptive social behavior
Features of more-advanced disease are as follows:
Muscle atrophy becomes more apparent
Spasticity may compromise gait and manual
dexterity
Muscle cramps are common
13. Treatment
Riluzole, decreasing the release of glutamate
and modifies the rate of progression
50 mg orally twice daily,
Symptomatic treatment:
Limb stiffness - antispasticity agents(baclofen,
tizanidine)
Pain – NSAIDs
Cramps - Quinine sulfate,benzodiazepines
14. Prognosis
Involvement of bulbar and respiratory muscles
accounts for most of the deaths in patients
with motor neurone disease.
Median survival – 3 years
15% will live 5 years after diagnosis
16. Primary lateral sclerosis
Disease of the voluntary muscle due to UMN
lesion
Symptoms: spasticity, weakness and stiffness
of legs, drop foot.
occurs spontaneously after age 50 and
progresses gradually over a number of year
17. Spinal Muscular Atrophy
autosomal-recessive disorders, characterized by
progressive weakness of the lower motor
neurons.
Related to defect on SMN1 gene on chromosome
5q
characterized by loss of anterior horn cells
Patients usually presenting with muscle weakness
and wasting in the limbs, respiratory, and bulbar
or brainstem muscles
The most common types are acute infantile (SMA
type I, or Werdnig-Hoffman disease), chronic
infantile (SMA type II), chronic juvenile (SMA type
III or Kugelberg-Welander disease), and adult
onset (SMA type IV) forms.