Amyotrophic lateral sclerosis, also known as ALS, is a rare but serious neurological disorder. It affects nerve cells in the brain and spinal cord, causing muscles to gradually become uncontrollable and unable to move. ALS usually leads to muscle atrophy and weakness, eventually causing the patient to lose mobility, speech, and even the ability to breathe.
The exact cause of ALS is unknown, but scientists believe that genetic, environmental, and lifestyle factors may all play a role in the development of the disease. Some studies have suggested that certain gene mutations may be involved in the development of ALS, but not all people with ALS have these gene mutations. In addition, some studies have found that environmental factors such as trauma or exposure to harmful substances may also increase the risk of developing ALS.
Symptoms of ALS usually appear in late middle age or early adulthood and may initially include mild weakness in the hands or feet, muscle stiffness, difficulty speaking or swallowing. As the disease progresses, these symptoms worsen, eventually leading to complete incapacitation. Imagine that you are doing your daily activities and suddenly realize that your arms or legs have become weak, as if they were frozen! This could be one of the early symptoms of ALS. As the condition worsens, you may find it increasingly difficult to express yourself verbally or even swallow food properly. It's like being frozen in ice, unable to move.
The most well-known example of this is the famous physicist Stephen Hawking, who was completely paralyzed by acromegaly at the age of 21, leaving him with only two eyes and three movable fingers. ALS severely affected his muscle control, causing him to be unable to walk, have difficulty speaking, and even rely on a wheelchair and voice synthesizer. However, Hawking is an extremely rare case of longevity amongst tardive dyskinesia patients. Most patients with ALS have a life expectancy of only 3-5 years, and those with severe disease may even have only a few months to live, with most dying of respiratory failure due to muscle atrophy and loss of respiratory muscle function.
There is no cure for ALS, but a number of medications and treatments can help slow the progression of the condition and manage symptoms. General treatment includes fall prevention care and nutritional support; for medication, riluzole, ropinirole and edaravone are commonly used. In addition, rehabilitation and supportive care are essential to improve the patient's quality of life.
Reference
Masrori, P., & Van Damme, P. (2020). Amyotrophic lateral sclerosis: a clinical review. European journal of neurology, 27(10), 1918-1929.
Feldman, E. L., Goutman, S. A., Petri, S., Mazzini, L., Savelieff, M. G., Shaw, P. J., & Sobue, G. (2022). Amyotrophic lateral sclerosis. The Lancet, 400(10360), 1363-1380.
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