Sclerosis als

Nucleus VII

VII nerve

Nucleus XI XI nerve

Decussation of corticospinal pathway

Lateral corticospinal tract

MEDULLA

SPINAL CORD

Anterior — horn cell

Nucleus VII

VII nerve

Nucleus XI XI nerve

MEDULLA

SPINAL CORD

Anterior — horn cell

Decussation of corticospinal pathway

Lateral corticospinal tract

Motor neuron disease (MND) is a progressive condition characterised by degeneration of upper and lower motor neurons.

Different terms are used to describe involvement at each level:

1. Frontal atrophy in the precental gyrus - frontal dementia

2. The corticobulbar pathway: pseudobulbar palsy.

3. The cranial nerve nuclei: progressive bulbar palsy.

4. The corticospinal tract: (Called primary lateral sclerosis when this tract alone is affected)

5. The anterior horn cell:

progressive muscular atrophy.

The term amyotrophic lateral sclerosis (ALS) is now used synonymously with motor neuron disease.

Epidemiology

Incidence: 2 per 100 000 per year, with a prevalence of 6 per 100 000. Clusters and conjugal cases have been reported.

Familial ALS accounts for 5% of cases and is usually inherited as a dominant trait. Sex ratio: male/female - 1.5:1 Mean age of onset - 55 years. Mean survival - 3 years (50%), over 5 yrs (28%)

neuron neuron

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