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What is ALS?

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Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease or Charcot’s Disease, is a degenerative condition of neuromuscular type by which some cells of the nervous system called motor neurons gradually decrease their functioning and die, producing progressive muscular paralysis of deadly prognosis, since in its late stages patients suffer total paralysis accompanied by the exaltation of tendon reflexes (resulting from loss of inhibitory muscle controls).

The name of the disease, first described in 1869 by French physician Jean Martin Charcot (1825-1893), specifies its main features:

“LATERAL SCLEROSIS”
Indicates the lost of nerve fibers accompanied by a “sclerosis” (from Greek, meaning “hardening”) or glial scarring in the lateral zone of the spinal cord, a region that is occupied by fibers or nerve axons which are the last responsible ones for the control of voluntary movements.

“AMYOTROPHIC”
It points out the muscular atrophy produced by chronic muscular inactivity, since muscles stop receiving nervous signals.

In ALS, brain functions not related with motor activity -i.e. sensibility and intelligence- remain unaltered.

On the other hand, motor neurons that control the eye’s extrinsic muscles are barely affected, for which patients conserve their ocular movements until the end. Likewise, ALS does not affect the Onuf nucleus, for which muscles of the sphincters that control urination and defecation are not affected either.

The disease specially affects people between 40 and 70 years of age, more frequently among men between 60 and 69 years of age. Each year, 2 cases every 100,000 inhabitants are produced