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Amyotrophic Lateral Sclerosis- Drug Today Medical Times

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Amyotrophic Lateral Sclerosis (ALS) is a rare neurological disorder with major involvement of the nerve cells or neurons. Neurons control the voluntary activities of the body such as chewing, walking and talking. The worldwide prevalence of this disease is about 1.9 per 100,000 people. It is also known as Lou Gehring’s disease named after the baseball player Lou Gehring who died of it.

ALS is a progressive disease, hence its symptoms will worsen over time. There is no cure for ALS as such till date and no treatment is available to stop, or reverse the disease progression. However, Riluzole (1995) and Radicava (2017) were approved for ALS treatment by the FDA to slow down the progression.

It is a motor neuron disease and causes gradual deterioration (degeneration) and death of the upper and lower motor neurons. Brain loses its ability to initiate and control voluntary actions of the body. ALS patients experience symptoms like muscle cramps, fasciculation, tight and stiff muscles, muscle weakness, slurred nasal speech and difficulty in chewing and swallowing. No exact cause of ALS is known. However, it has been attributed to be due to genetic and environmental factors.

Early symptoms of ALS appear as weakness or stiffness. Eventually, all the voluntary muscles get affected and the patient loses his/her strength and ability to speak, eat, move and even breathe. Most of the patients die from respiratory failure, usually within 3 to 5 years of disease diagnosis. However, around 10 percent of people survive for 10 years or more, as seen in the case of famous cosmologist Stephen Hawking who survived for 55 years after he was first diagnosed with ALS.

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