Amyotrophic lateral sclerosis is an incurable and fatal disease of the nervous system. The unstoppable damage to nerve cells is accompanied by progressive paralysis of all muscles.

Synonyms

ALS, Myatrophic lateral sclerosis, Motor neurone disease, Lou Gehrig syndrome, Charcot disease.

Definition

Amyotrophic lateral sclerosis (ALS) is a very rare and incurable disease of the nervous system. In the English-speaking world it is referred to as Motor Neurone Disease. This name reflects the cause of the disease. In amyotrophic lateral sclerosis, the nerve cells responsible for the excitation of the muscles degenerate. These nerve cells are called motor neurons. In public, ALS is sometimes confused with multiple sclerosis (MS) . In fact, there are very different clinical pictures.

The world’s best known patient with ALS is physicist Stephen Hawking. He was diagnosed with slow-moving juvenile ALS at the age of 21.

Frequency

The frequency of ALS is given with a maximum of 3 new cases per year and 100,000 inhabitants (annual incidence). This makes amyotrophic lateral sclerosis one of the rare diseases, orphan diseases. According to estimates, between 3,000 and 4,000 people with amyotrophic lateral sclerosis live in Germany. Men are affected a little more often than women. In most cases, the disease breaks out between the ages of 50 and 70.

Symptoms

Amyotrophic lateral sclerosis (ALS) is associated with progressive spasticity with muscle spasms and paralysis and the associated muscle breakdown. Muscle wasting usually begins in the arms and hands and spreads to the feet. The course can also be very different.

Early symptoms of ALS are primarily muscle twitches and impairments in the mouth and tongue. People with ALS often have problems speaking, chewing, and swallowing.

Course

Amyotrophic lateral sclerosis is always fatal. Most sufferers die 3 to 5 years after diagnosis. With juvenile ALS, the survival time can be significantly longer. But here, too, the disease is fatal. Pneumonia, which results from paralyzed breathing muscles and swallowing disorders, is often the cause of death.

Causes

The causes of amyotrophic lateral sclerosis have not yet been clearly clarified. A familial clustering of the disease can be observed in about 15 percent of the cases. There are also regions in which ALS occurs up to 50 times more frequently than usual, for example in Guam. Gene mutations can be detected in almost all those affected. So far it is not clear whether these changes actually lead to the onset of the disease. It is also quite 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.

Treatment

Amyotrophic lateral sclerosis is not curable. Symptomatic treatment focuses on alleviating symptoms, controlling complications and maintaining the greatest possible quality of life for those affected.

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