Amyotrophic lateral sclerosis, better known by its abbreviation ALS, is a rare, incurable nervous disease. It causes paralysis of the muscles all over the body, which is why those affected are dependent on a wheelchair at an early stage. The disease ends fatally.
ALS is a degenerative (from degenerate = decay) Disease of the central nervous system. The nerve cells responsible for movement degenerate and can no longer give impulses to the muscles. Those affected gradually experience paralysis of the entire musculoskeletal system, and are usually dependent on a wheelchair a few months after the diagnosis. Bit by bit they lose the ability to walk, lose their facial expressions, sooner or later they can no longer put their forks to their mouths on their own. At the same time, involuntary twitching of the muscles, so-called fasciculations, occurs. Eventually, those affected can no longer swallow or breathe, which is why amyotrophic lateral sclerosis inevitably leads to death.
Frequency of ALS in the population
According to the German Society for Muscle Diseases (DGM), around one to two per 100,000 people worldwide develop ALS every year, mostly between the ages of 50 and 70. Men are affected slightly more often than women and Europeans slightly more often than people in the rest of the world.
forms of amyotrophic lateral sclerosis
Possible first symptoms
In some patients, an apparently inconspicuous muscle weakness, often initially only in one hand, is the first symptom, others complain of muscle cramps, and in still others the ability to speak degenerates first. As a rule, muscle paralysis begins in the extremities and progresses to the face.
Possible first symptoms at a glance:
- muscle twitching
- muscle cramps
- Muscle weakness in one hand/arm
- Muscle weakness in a lower leg/foot
- clumsiness
- Unsteady gait
- Trouble speaking, chewing, or swallowing
- Spastic paralysis
How is amyotrophic lateral sclerosis diagnosed?
In order to diagnose amyotrophic lateral sclerosis, a detailed medical history is taken, and the nerve conduction velocity is examined at the same time. Other tests follow as standard (including nerve function, blood and lung function tests and magnetic resonance imaging) in order to be able to rule out other diseases that the symptoms of ALS can resemble.
What does the treatment look like?
If the suspicion is confirmed, regular monitoring of the course of the disease should be carried out by a neurologist who specializes in amyotrophic lateral sclerosis. Unfortunately, the development cannot be stopped, only monitored. In order to be able to deal better with the fact that more and more bodily functions are lost in the medium term, ALS patients should seek occupational, physiotherapeutic and speech therapy treatment. Psychotherapeutic care should not be underestimated. Furthermore, as the disease progresses, those affected are increasingly dependent on technical aids to communicate, breathe and move.
Are there risk groups?
According to the current state of science, ALS usually occurs spontaneously. A mutation of a specific metabolic gene can only be found in about every hundredth person affected, which is therefore unable or less able to absorb free metabolic radicals, which can promote nerve damage.
It is striking that athletes are often affected. This has been shown again and again in prominent individual cases and has also been shown in studies. However, scientists can (so far) only speculate about the causes of this increased risk. A possible explanation could be genetic factors and also that contact sports can increase the incidence of head injuries and this can promote the onset of the (sometimes genetic) disease.1,2
Krzysztof Nowak (†2005), former Polish national player and ex-professional at Bundesliga club VfL Wolfsburg, like several other athletes fell ill with ALS – and diedPhoto: Getty Images
What is the prognosis for amyotrophic lateral sclerosis?
The speed at which the disease progresses varies. On average, the patients live about two and a half years when the first symptoms are noticed, 10 to 20 percent of the patients even live more than ten years.3,4 Important: The mental abilities of the patients are mostly retained completely. This means that they are fully aware of the decline in all motor skills. The sensory and digestive organs also usually function until death.
Even if eating (swallowing) is becoming increasingly difficult for those affected, it is important that they do not lose too much weight – this would further accelerate physical deterioration. Conversely, being slightly overweight and having a moderately high cholesterol level can at least increase life expectancy somewhat, as scientists at Massachusetts General Hospital have determined in an analysis of patient data.5
Can ALS be inherited?
According to the current state of science, heredity does not appear to play a role in the development of ALS. In 90 to 95 percent of cases, a family predisposition to ALS cannot be identified. Familial clustering occurs in 5 to 10 percent.6
FITBOOK received professional advice from general practitioner and emergency physician Dr. Falk Stirkat.
Sources
- 1. Julian, Thomas & Glascow, Nicholas & Barry, A & Moll, Tobias & Harvey, Calum & Klimentidis, Yann & Newell, Michelle & Zhang, Sai & Snyder, Michael & Cooper-Knock, Johnathan & Shaw, Pamela. (2020). Physical exercise is a risk factor for amyotrophic lateral sclerosis: convergent evidence from mendelian randomisation, transcriptomics and risk genotypes. 10.1101/2020.11.24.20238063.
- 2. Blecher R, Elliott MA, Yilmaz E, Dettori JR, Oskouian RJ, Patel A, Clarke A, Hutton M, McGuire R, Dunn R, DeVine J, Twaddle B, Chapman JR. Contact Sports as a Risk Factor for Amyotrophic Lateral Sclerosis: A Systematic Review. Global Spine J. 2019 Feb;9(1):104-118. doi: 10.1177/2192568218813916. Epub 2019 Jan 31. PMID: 30775214; PMCID: PMC6362556.
- 3. Traxinger K, Kelly C, Johnson BA, Lyles RH, Glass JD. Prognosis and epidemiology of amyotrophic lateral sclerosis: Analysis of a clinic population, 1997-2011. Neurol Clinic Practice. 2013;3(4):313-320. doi:10.1212/CPJ.0b013e3182a1b8ab
- 4. Chiò A, Logroscino G, Hardiman O, et al. Prognostic factors in ALS: A critical review. Amyotrophic Lateral Scler. 2009;10(5-6):310-323. doi:10.3109/17482960802566824
- 5. Paganoni S, Deng J, Jaffa M, Cudkowicz ME, Wills AM. Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis. muscle nerves. 2011 Jul;44(1):20-4. doi: 10.1002/mus.22114. Epub 2011 May 23. PMID: 21607987; PMCID: PMC4441750.
- 6. Zarei S, Carr K, Reiley L, et al. A comprehensive review of amyotrophic lateral sclerosis. Surg Neurol Int. 2015;6:171. Published 2015 Nov 16. doi:10.4103/2152-7806.169561
Other sources