31 ALS

Question 1

Define amyotophic lateral sclerosis

Answer 1

Part of a group of disorders called motoneurone diseases. Causes motoneuron degeneration affecting UMN in cerebral cortex, brain stem, and spinal cord.

Question 2

Epidemiology of ALS

Answer 2

5/100000
90% have no family hx
average onset 5th decade
Death usually within 2-5 years

Question 3

Symptoms of ALS

Answer 3

Progressive wasting, weakness and atrophy of muscles leading to paralysis.
Dysphagia, dysarthia
Impairment of respiration
Exaggerated reflexes and increased muscle tone (spasticity)
Signs of muscle denervation (fibrillations, fasciculations)
No involvement of extraoccular muscles/shpincters
No sensory or intellectual deficits.

Question 4

Pathogenesis of ALS

Answer 4

Autoimmune hypothesis
Neurotropic hypothesis
Oxidative stress hypothesisi
FAS induced cytotoxicity
Excitotoxic hypothesis