Amyotrophic Lateral Sclerosis overview

ALS (Amyotrophic Lateral Sclerosis, which is also known as Lou Gehrig’s disease) is a progressive, fatal neuromuscular disease that affects nerve cells in the brain and spinal cord, eventually leading to a loss of muscle control and paralysis. ALS prevents motor neurons sending impulses from the brain and spinal cord to the muscles, causing muscle atrophy and weakness. Voluntary muscle action is progressively lost and in late-stage ALS patients may have difficulty communicating, swallowing, moving, and breathing. There is currently no cure for ALS.

Cachexia may occur in the early stages of ALS, has a significant impact on quality of life and can be terminal in some patients1.

It is estimated that in 5-10 percent of ALS cases there is a clear genetic history but in the majority of patients there is no clear identifying genetic or environmental cause for the disease. In most cases ALS does not impair a person’s sensory functions (vision, hearing, taste, sense of smell and touch) or intellectual reasoning. However, a large percentage of people with ALS do develop some degree of cognitive or behavioural impairment2.

Average life expectancy following diagnosis is 2-5 years and, in the US alone, over 5000 people are diagnosed with the disease each year, where it is estimated that the out-of-pocket cost for caring for a person with ALS is $250,0003.

1Causes of death in amyotrophic lateral sclerosis : Results from the Rhineland-Palatinate ALS registry J Wolf 1, A Safer 2, J C Wöhrle 3, F Palm 4, W A Nix 5, M Maschke 6, A J Grau 4 PMID: 28184974 DOI: 10.1007/s00115-017-0293-3

2Crockford, C., Newton, J., Lonergan, K., Chiwera, T., Booth, T., Chandran, S., Colville, S., Heverin, M., Mays, I., Pal, S., Pender, N., Pinto-Grau, M., Radakovic, R., Shaw, C. E., Stephenson, L., Swingler, R., Vajda, A., Al-Chalabi, A., Hardiman, O., & Abrahams, S. (2018). ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS. Neurology, 91(15), e1370–e1380.

3ALS Association website What is ALS? | The ALS Association