Amyotrophic lateral sclerosis (ALS)

August 2017


Definition


Amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, is a rare progressive degenerative disease that affects motor neurons. The origin is unknown, but it leads to a deterioration of neurons responsible for the transfer of the order of movement to motor neurons. These neurons are affected in the central nervous system, brain and spinal cord, but also the peripheral nerves. Typically, it affects adults between the ages of 40 and 70. This leads inexorably to the death of nerve cells that allow for the movement of voluntary muscles, and consequently paralysis. Its course is variable from one subject to another, but typically, death occurs within a few years. At present, there is no treatment that can stop the progression of the disease or cure ALS.

Symptoms


The patient with amyotrophic lateral sclerosis can experience various symptoms of progressive evolution, frequently starting at the upper limbs and extending progressively:
  • muscle cramps;
  • difficulties in movement, progressing to paralysis;
  • decrease in muscle mass, known as atrophy;
  • slowness of movement;
  • involuntary muscle contractions in the form of twitching;
  • swallowing disorders, feeding difficulties;
  • speech problems

Diagnosis


Additional tests such as electromyography, which allows to study the spontaneous activity of muscles and their response to stimulation, as well as muscle biopsy (of an affected muscle), allow to strengthen the suspicion. The latter are usually complemented by a review of brain imaging and blood tests.

Treatment


Several axes are needed for the treatment of amyotrophic lateral sclerosis. The disease is currently not curable, and so the treatment of symptoms is essential to lessen the impact on the individual and to enable a more optimal quality of life. A molecule is used and has shown some efficacy in the disease: riluzole. Studies are underway to find other therapeutic techniques. Parallel to drug therapy, physiotherapy and a medico-social care with the implementation of technical assistance and psychological counseling are essential.

Related

Published by Jeff. Latest update on July 23, 2013 at 11:41 AM by Jeff.
This document, titled "Amyotrophic lateral sclerosis (ALS)," is available under the Creative Commons license. Any copy, reuse, or modification of the content should be sufficiently credited to CCM Health (health.ccm.net).