A myotrophic lateral sclerosis first became a sensation in the United States via American Baseball player Lou Gehrig who represented the New York Yankees. He was the first MLB player to have his uniform number retired after his farewell speech. After two years, Lou Gehrig died in his sleep in his New York home on June 2, 1941, due to the fatal disease. Since then, his name became synonymous to Amyotrophic lateral sclerosis across North America.
he well-known scientist Stephen Hawking is rarest of the rare not only in the world of physics but also in the world of ALS; When most people with the disorder on an average die within 3 years from diagnosis, the theoretical physicist has survived nearly five decades from being diagnosed with the degenerative motor neuron disorder.
Mr. Hawking’s longevity has been due to the excellent care he is been offered for the last five decades. However, one should understand the nature and type of neurodegenerative disease are the determinants of the patients’ lifespan. The complexity lies in the variability of the disorder but only a small fraction of people diagnosed with this form of dementia live a long life.
The disorder affects the upper motor neurons (brain) or the lower motor neurons (Spine) and in some cases both. It is important to understand each muscle in the body is controlled by motor neurons that reside in the frontal regions of the brain. Medically, all neurodegenerative diseases including Alzheimer’s and Parkinson’s share important traits such as spreading of toxic proteins from one neuron to another, inflammation of neurons, the decrease in the recycling of misfolded proteins and increase in the amount of protein misfolding.
Pathologically, evidence of deteriorating upper motor neurons has been found in autopsy procedures despite scientific community often seeing progressive muscular atrophy (a form of ALS) as isolated illness of the lower motor neurons. Resources need to be poured and the definitions need to be recreated if problems have to be addressed.
As such there is no treatment for the disorder but the quality of life could be improved with proper medication for memory loss, treatment for behavioral changes and change in sleep patterns. The disorder is quite fatal when the motor neurons running the diaphragm degenerates often resulting in respiratory failure. Also, deterioration of swallowing muscles leads to malnutrition and dehydration. Other than these cases, patients could live longer but the condition is expected to get worse over time as toxic proteins pass from one neuron to another.
Due to the absence of a cure, the disorder cannot be reversed but largely medical research is focused on slowing the progression of symptoms and complications. The survival and quality of life largely depend on the quality of assistance provided as in the case of Stephen Hawking. Medicines used in the treatment of muscle cramps, constipation, fatigue, pain, depression and sleep problems are used to suppress symptoms throughout patient’s life from time to time.