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In 1993, a group of geneticists and clinicians discovered a gene responsible for one form of hereditary Lou Gehrig’s diseases that represents almost 2 percents of all cases. With the increase in the rate of the ALS disease, there is a need to review the causes, signs and symptoms, diagnosis, management and treatment of the disease. In 1939, the New York Yankees first baseman was diagnosed with Lou Gehrig’s disease. The baseman died two years later due to progressive neuromuscular disorder.
In 1869, Jean-Martin Charcot who was a clinician indicated that the disease was misunderstood. Some doctors thought that the disease was a rare one, but today they consider it to be fairly common (Muscular Dystrophy Association Inc, 12) Signs and Symptoms Lou Gehrig’s disease is exemplified by the weakening of both the upper and the lower motor neurons. On the onset stage, the symptoms of the disease are often overlooked. Some of the early symptoms include twitching, cramping of muscles becoming stiff, muscle weaknesses that generally affects the arms or legs, slurred and nasal speech.
Others may experience difficulties in chewing or swallowing (Jerry, 22). These symptoms may later develop into atrophies which may cause a physician to suspect Lou Gehrig disease. The body parts that are affected by the early symptoms mainly depend on motor neurons that are lost first or are damaged. When atrophies spread to other body parts the patients have problems in moving, swallowing, speaking, forming vocabulary. When patients with ALS complain of tight and stiff muscles and exaggerated reflexes that may include an overactive gag reflex, it means that the upper motor neurons are affected.
The upper motor neurons are located on the surface of the brain and exert power over the lower motor neurons, located in the brainstem and the spinal cord (Jerry, 20). When the lower motor neurons degenerate the symptoms include muscle weaknesses and withering, muscle spasms, and momentary contraction of muscles which are noticeable under the skin. The lower motor neurons are attached to muscles through axons. These bundles of the axons leave the spinal cord and move to the muscles. Lou Gehrig’s disease is diagnosed through a series of clinical examinations and diagnostics.
Clinical diagnosis highly depends on the patient’s history, physical examination, laboratory and radiographic evaluation related to ALS. Patients erythrocyte sedimentation rate, serum and urine protein electrophoresis are measured. Other tests conducted to establish the diagnosis include thyroid function tests, serum calcium and phosphate measurements, and cerebrospinal fluid analysis are done. Electromyography may also be used to identify loss of lower motor neurons (Antao and Horton, 5) Treatment Currently there has been no definite cure for ALS.
Effective symptomatic management and improving the quality of life have been established as the primary goals in Lou Gehrig disease patients care (Antao and Horton, 7). However, Rilutek (Riluzole) drug was accepted by the Food and Drug Administration for the treatment of the disease. The drug is thought to reduce the damage on motor neurons by decreasing the release of glutamate. It also increases the patient’s rate of survival and may extend the period until which a patient may need ventilatory support (Jacoby and Youngson, 84).
However, Ritulek does not reverse any
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