The Disease - ALS:
Amyotrophic lateral sclerosis belongs to the group of motor neuron diseases.
This group of motor neuron diseases is completed by the spinal muscular atrophy, a special form is the Kennedy disease. ALS is historically seen as a condition that manifests itself in motor symptoms. This in turn, is due to the fact that a disease of the 1st motoneuron (the Betz cell and the corticospinal tract) of the 2nd motor neuron (the anterior horn cell and its axon) is in the foreground of the clinical deficits. This picture, which Charcot has already drawn, has given way to a much more complex scenario today. With an age peak of 55 to 75 years, there are motor deficits in the extremities or in the cranial nerve nuclei in the sense of paralysis. The disease process then spreads continuously throughout the body. At the beginning of the disease are often convulsions and fasciculations, sometimes an unexplained weight loss; the characteristic amyotrophies and paralyzes follow. According to our epidemiological findings, this disease will affect every 400th German.
Therapeutically, there is currently only one drug that slows the disease down. The therapeutic effect in the pivotal study was about 1 year with a life expectancy of about 1 year and an extension of life by 3-4 months. However, many studies, which were retrospective, report a better effect of Rilutek (Riluzole) when treated earlier. Therapeutic focus is on the supply of aids, support of the respiratory function (not only the ventilation) as well as ensuring the nutrition. It must be ensured by sufficient calorie intake that the patient does not unnecessarily lose weight (catabolic), as this is a negative prognostic factor. The supply of nutrition can be achieved on the one hand by high-calorie supplemental nutrition, on the other hand by the installation of a percutaneous endoscopic gastrostomy (PEG).
Many people think that Amyotrophic Lateral Sclerosis (ALS) is a rare disease. However, the risk of developing ALS and dying is around 1: 400 in Germany. Over the age of 50, this risk is considerably higher. 200,000 of the currently 80 million inhabitants in Germany will die of an ALS.
(Source: Charcot Foundation)