MOTOR SYSTEM DISEASE

Chronic motor neurone disease, Progressive Muscular Atrophy, MND)

MOTOR SYSTEM DISEASE are with progressive chronic idiopathic degeneration of the motor neurons in the cerebral cotex, brain stem and spinal cord resulting in gradual atrophy, weakness and paralysis of muscles with fasciculation. Sensory and cerebellar dysfunctions are however absent. Sphincters are not affected. About 5% of cases are familial with autosomal dominant inheritance. The remaining 95 % of cases are having various unproved possible causes.

CLINICAL FEATURES

Onset is insidious.

Age: 40-60 years. Rare under 20 years.

Sex: Males are commonly affected.

Clinically the following types of presentation are seen.

1. Progressive muscular atrophy or True atrophic type of PMA.

2. Amyotrophic lateral sclerosis.

3. Chronic bulbar palsy.

4. Pseudobulbar palsy.

5. Primary lateral sclerosis.

6. Progressive spinal muscular atrophy in children (Werdnig-Hoffmann paralysis).

7. Neural form of peroneal muscular atrophy (Charcot-Marie-Tooth disease).

8. Kugelberberg-Weilander syndrome.

First, third and fourth may appear alone for many months but first and second often appear together.

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