Motor neurone disease د.رشاد عبدالغني

8/3/2019 Motor neurone disease .

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Motor neurone disease

The various forms of adult motor neurone diseaseare classified according to the distribution of the

initial clinical features


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The clinical findings are the consequence of motor neurone

loss in the cortex, brain stem and spinal cord, along withdegeneration in the corticobulbar and corticospinal pathways.

Pathology


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In progressive muscular atrophy

Weakness and wasting of the small hand muscles begin

asymmetrically then extend more uniformly over severalyears. Reflexes are relatively preserved, despite the wasting,

and fasciculation is prominent. Initial involvement of the

lower limbs is less common, presenting usually with foot drop

or wasting of one or both thighs.


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The spinal muscular atrophies (SMAs)

They differ in age at onset and severity of

symptoms.

SMA type I (severe form): Onset is from birth to 6

months. Children are never able to sit without support, and

death usually occurs before the age of 2 years.SMA type II (intermediate form): Onset is before the age

of 18 months. Children are unable to stand or walk without

aid, and death usually occurs after the age of 2 years.

SMA type III (mild form): Onset is after the age of 18

months. Patients have the ability to stand and walk, and

death occurs in adulthood.


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Primary lateral sclerosis is rare. Here there is a

slowly progressive spastic paraparesis with or

without upper limb involvement and without motorneurone loss.

In amyotrophic lateral sclerosis, there is a

combination of spasticity and hyperreflexia in thelower limbs with weakness, wasting and

fasciculation in the upper limbs. Later in the illness

truncal and oropharyngeal muscles are affected.


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Progressive bulbar palsy presents with dysphagia and

dysarthria. The facial muscles atrophy, speech becomes

slurred and aspiration is likely. Weakness and wasting of the

tongue become conspicuous, accompanied by fasciculationand slowing of movement.

include sensory impairment, sphincter

disorders and ocular involvement.

Exclusion criteria


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Lab studies

EMG studies are of considerable value indiagnosis. A combination of fibrillation and

fasciculation potentials with large, prolonged

motor unit potentials is particularly characteristic


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Muscle biopsy shows the changes of chronic

denervation with grouped fibre atrophy and fibre-

type grouping without evidence of inflammatorycell infiltration

Normal muscle contain

random checkerboard-like appearance

Fibre-type grouping
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Medical care in ALS is primarily palliative.

Patients should be involved in regular exercise

and a physical therapy program.

Medications such as baclofen and tizanidinemay be used to relieve severe spasticity.

Riluzole, a glutamate inhibitor, is an FDA-

approved medication for prolongingtracheostomy-free survival.

Treatment:


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Syringomyelia

In syringomyelia cystic cavitation of the spinal cord occurs,

most prominently in the cervical region sometimes inassociation with cystic cavitation in the brain stem

(syringobulbia).


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Causes:

The condition closely coexists with a number of

developmental anomalies close to the cervicocranial

junction, including abnormal fusion of the cervical

vertebrae, Type 1 Chiari malformation.

Syringomyelia may also appear in relationship to anintramedullary tumour, and as a post-traumatic

phenomenon.


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Clinical features

There is prominent dissociated anaesthesia in the cervicaldermatomes, often leading to injuries to the hands.


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Later, touch and proprioceptive function may be affected.

The motor involvement includes weakness then wastingin upper limb muscles and a spastic paraparesis.


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The upper limb reflexes become depressed, the lower limb

reflexes exaggerated. Autonomic dysfunction occurs and

includes a Horners syndrome, altered sweating of the face

and arms, and sphincter disturbance. Kyphoscoliosis is

sometimes found.


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Investigation

CT myelography can demonstrate expanded cord and

delayed opacification of the syringomyelic cavity


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MRIis the procedure of choice,being more accurate in delineating

the extent of cavitation and thecerebellar herniation

Cavity


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Various forms of surgical treatment have been used

including: foramen magnum decompression and

syringoperitoneal or subarachnoid shunting.

Treatment

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Motor neurone disease د.رشاد عبدالغني