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Name of Disease: Amyotrophic lateral sclerosis (ALS) or Lou Gehrig’s Disease

Root cause of disease: The root cause of ALS is unknown although 1 out of 10 cases is due to a genetic defect.

Affected cell types/tissues/organs/systems: Nerve cells are the primary affected cell types, but muscle cells are also affected as the nerves waste away and die [1].

Historical Background:
Charles Bell first mentioned ALS in a report in 1824, and in 1850 Augustus Waller described the appearance of shriveled nerve fibers in the body. A French doctor names Jean-Martin Charcot described ALS in scientific literature. On July 4, 1939 ALS gained national notoriety when Lou Gehrig gave his farewell speech from both the New York Yankees and baseball due to his diagnosis with ALS. Numerous charities have been formed to raise money to fight this disease in his name. With his diagnosis of ALS the disease also became known as Lou Gehrig’s disease. Other famous individuals with amyotrophic lateral sclerosis consist of the famous physicist Stephen Hawking as well as the first leader of China’s communist party Mao Zedong.

Common Symptoms:
Common symptoms for people suffering from ALS consist of a loss of muscle strength and coordination. This is due to the nerve cells in their body wasting away and dying which in turn prevent muscles from being properly stimulated which then leads to their degradation. The muscles associated with breathing and swallowing are typically the first muscles affected by ALS. As the disease progressed the other muscle groups are affected as well. Other common symptoms include difficulty breathing, difficulty swallowing, head drop due to weakness of the neck muscles, muscle cramps, muscle contractions, paralysis, speech problems, voice changes and weight loss [1].

Standard Treatments:
There is no known cure for ALS. Although there is a medicine that slows down the disease and allows for an individual to live longer, this drug is called riluzole. Other methods of treatment consist of drugs to reduce muscle spasms, and to assist people with problems swallowing their own saliva. Physical therapy is also commonly used in order to maintain a person’s muscle function and their general health. Some patients may decide to have a feeding tube placed into their stomach as the disease worsens so that they do not choke on their food. It is also important for a nutritionist to get involved in treating somebody with ALS to ensure that they get all of the nutrients they nee. Lastly breathing devices are used at night to ensure that a person is always breathing while they are asleep [1].

Current Research:
Research was done into how amyotrophic lateral sclerosis progresses throughout the body and affects the neurons. It was found that cerebrospinal fluid has a cytotoxicity that allows for the disease to progress. It was determined that the glutamate receptors in cells where they have a role in ion channels of nerve cells. [2]

Commonly a person suffering from amyotrophic lateral sclerosis is accompanied with cognitive or behavioral impairments. Typically these changes are associated with frontotemporal dementia. Current neuropathological findings suggest that amyotrophic lateral sclerosis does not have an affect on the frontotemporal network in a person’s brain. The researchers go on to say that instead amyotrophic lateral sclerosis has an overall affect on the person’s brain, which fits with what the disease is. [3] With the person’s nerves being degraded all over.

Research being done on possible treatment for people suffering from amyotrophic lateral sclerosis tends to be more along the lines of making life easier. This is because the cause for the disease is unknown so a true cure for it cannot be yet developed. Technology-aided programs are very helpful to people suffering from amyotrophic lateral sclerosis. These programs allow people to send text messages to people, as well as to communicate with other people through voice assistance programs. New technologies are being developed all the time in order to make life as normal as possible for people with ALS. [4]

References:
[1] Amyotrophic lateral sclerosis (2012) U.S. National Library of Medicine: Diseases and Conditions.

[2] Matias-Guiu, J.; Galan, L.; Garci-Ramos, R.; Barcia, J.A.; Guerrero, A. (2010). Cerebrospinal fluid cytotoxicity in lateral amyotrophic sclerosis. Neurologia 6.

[3] Tsermentseli, Stella; Nigel Leigh, P.; Goldstein, Laura (2012) The anatomy of cognitive impairment in amyotrophic lateral sclerosis: More than frontal lobe dysfunction. Cortex 2.

[4] Lancioni, Giulio; Singh, Nirbhay; O’Reilly, Mark; Sigafoos, Jeff; Ferlisi, Gabriele; Ferrarese, Giacomina; Zullo, Valeria; Addante, Luigi; Spica, Antonella; Oliva, Doretta (2012). Technology-aided programs for assisting communication and leisure engagement of persons with amyotrophic lateral sclerosis: Two single-case studies. Research in Developmental Disabilities 5.

Alberts, Bruce; Johnson, Alexander; Lewis, Julian; Raff, Martin; Roberts, Keith; Walter, Peter (2008). Molecular Biology of The Cell. Garland Science Taylor & Francis Group, New York.