Amyotrophic lateral sclerosis is an incurable. Fatal disease of the nervous system. The inexorable damage to the nerve cells is accompanied by progressive paralysis of all muscles.
ALS, myatrophic lateral sclerosis, motor neurone disease, Lou Gehrig's syndrome, Charcot disease.
Amyotrophic lateral sclerosis (ALS) is a very rare incurable disease of the nervous system. It is called motor neurone disease in the English-speaking world. This designation reflects the cause of the disease. In amyotrophic lateral sclerosis, the nerve cells responsible for excitation of the muscles atrophy (degenerate). These nerve cells are called motor neurons or motoneurons. In public, ALS is sometimes confused with multiple sclerosis (MS). In fact, they are very different clinical pictures.
The world's most famous patient with ALS is the physicist Stephen Hawking. He was diagnosed with slow-onset juvenile ALS at the age of 21. Hawking died in 2018 at the age of 76.
The incidence of ALS is estimated at a maximum of 3 new cases per year and 100 cases per year.000 inhabitants (annual incidence). This makes amyotrophic lateral sclerosis a rare disease, or orphan disease. In Germany, it is estimated that between 3.000 and 4000 people with amyotrophic lateral sclerosis. Men are affected slightly more often than women. In most cases, the disease breaks out between the age of 50 and the age of onset. and 70. year of life from.
Amyotrophic lateral sclerosis (ALS) is accompanied by progressive muscle spasms as well as paralysis and an associated muscle degeneration. Typically, muscle loss begins in the arms and hands and spreads to the feet. However, the course of the disease can also be quite different.
Early symptoms of ALS are mainly muscle twitching and impairments in the area of the mouth and tongue. People with ALS often suffer from problems with speaking, chewing and swallowing.
Amyotrophic lateral sclerosis is always fatal. Most sufferers die 3 to 5 years after diagnosis. In juvenile ALS, the survival time can be significantly longer. An example of this is the British physicist Steven Hawking (died at 76) reach old age. Ultimately, however, juvenile ALS is also fatal. Cause of death is often pneumonia, which occurs as a result of paralyzed respiratory muscles and swallowing disorders.
The causes of amyotrophic lateral sclerosis are not yet clearly understood. In ca. A familial clustering of the disease can be observed in 15 percent of cases. In addition, there are regions where ALS is up to 50 times more common than usual, for example on Guam.
Gene mutations can be detected in almost all affected individuals. However, it is not yet clear whether these changes actually lead to the onset of the disease. It is also possible that sports injuries to the head and chemicals such as chlorinated hydrocarbons from pesticides or biphenyls from plastics and textiles can promote amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis is not curable. Symptomatic treatment focuses on relieving symptoms, managing complications, and preserving as much quality of life as possible for affected individuals.