Definition:
Lou Gehrig's Disease, also called Amyotrophic Lateral Sclerosis or ALS, is a progressive and often fatal neurodegenerative disorder characterized by motor neuron degeneration. Motor neurons are nerve cells in the central nervous system that are primarily responsible for voluntary muscle movement. When these neurons degenerate, the resulting disease is known as Lou Gehrig's.
Treatment:
Currently, no known cure has been found to treat Lou Gehrig's Disease. Recently, however, a new drug treatment has been approved for the disease: Riluzole (Rilutek). This medication is expected to decrease the release of glutamate, thus leading to a significant reduction of the motor neuron damage. Clinical trials have shown that Riluzole increases survival rate among patients.
Other treatment strategies for Lou Gehrig's disease focus mainly on alleviating symptoms and improving the quality of life of afflicted individuals.
Symptoms and Signs:
As a neurodegenerative disorder, Lou Gehrig's disease leads to muscle weakness and atrophy throughout the body. When the upper and lower motor neurons degenerate, they stop sending messages to the muscles. As a result, the muscles are unable to function, gradually weaken, develop fasciculations, and gradually atrophy because of denervation. Ultimately, afflicted patients may completely lose the ability to control all voluntary movements excepting eye functions.
Lou Gehrig's disease usually spares cognitive functions. However, in certain cases where ALS is correlated with frontotemporal dementia, cognitive function may be compromised as well.
Afflicted patients may demonstrate only mild symptoms at the onset of the disease. Twitching, muscle stiffness, cramping, muscle weakness, and a slurring of speech are early signs. From these general symptoms, more obvious weakness or atrophy may cause a suspicion of ALS.
To be diagnosed with Lou Gehrig's disease, afflicted patients must demonstrate symptoms of both upper and lower motor neuron damage that cannot be linked to other causes.
Causes:
Familial ALS results from a number of genetic factors. This disease has been associated with a mutation of the copper/zincSuperoxide dismutase (SOD1), a type of enzyme responsible for hunting free radicals. Many believe that the mutation is inherited via autosomal dominancy.
To date, no definitive cause for Lou Gehrig's disease has been identified. However, it has been linked to such factors as viral infections, neurotoxin exposure, DNA defects, abnormalities in the immune system, and enzyme defects.
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