WHAT IS ALS?

Amyotrophic lateral sclerosis (ALS) or Lou Gehrig's disease, and rarely Charcot disease—is a neurodegenerative disorderwith various causes. The term motor neurone disease (MND) is sometimes used interchangeably with ALS while others use it to refer to a group of similar conditions that include ALS.  ALS is characterised by muscle spasticity, rapidly progressive weakness due to muscle wasting. This results in difficulty speaking, swallowing, and breathing. The disease usually starts around the age of 60, except in cases that are directly inherited when the usual age of onset is around 50.

About 5 to 10% of cases are directly inherited from a person's parents. ALS is the most common of the five types of motor neuron disease.

The average survival from onset to death is three to four years.  Only 4% survive longer than 10 years, although rare cases survive 50 years or more. Most die from respiratory failure. In much of the world rates of ALS are unknown. In Europe the disease affects about 2.2 people per 100,000 per year. In the United States, more than 5,600 are diagnosed every year, and up to 30,000 Americans are currently affected. ALS is responsible for 2 deaths per 100,000 people per year.

Descriptions of the disease date back to at least 1824 by Charles Bell. In 1869 the connection between the symptoms and the underlying neurological problems were first described by Jean-Martin Charcot who in 1874 began using the term amyotrophic lateral sclerosis. It became well known in the United States when it affected a famous baseball player by the name of Lou Gehrig, and later when the ice bucket challenge became popular in 2014.