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Amyotrophic Lateral Sclerosis

What is Amyotrophic Lateral Sclerosis ?

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease of muscular atrophy. It's also known as Lou Gehrig's disease (after a well-known baseball player who died of the disease in 1941). ALS is a chronic, progressive, and debilitating disease that is invariably fatal. It's characterized by progressive degeneration of the anterior horn cells of the spinal cord and cranial nerves and of the motor nuclei in the cerebral cortex and corticospinal tracts.

ALS is about three times more common in men man in women. Generally, ALS affects people ages 40 to 70. Most patients with ALS die after about 3 years, but some may live as long as 10 to 15 years. Death usually results from a complication, such as aspiration pneumonia or respiratory failure.

Causes of Amyotrophic Lateral Sclerosis

The exact cause of ALS is unknown, but about 10% of ALS patients inherit the disease as an autosomal dominant trait. ALS may also be caused by a virus that creates metabolic disturbances in motor neurons or by immune complexes such as those formed in autoimmune disorders.

Precipitating factors that can cause acute deterioration include severe stress, such as myocardial infarction, traumatic injury, viral infections, and physical exhaustion.

Signs & Symptoms of Amyotrophic Lateral Sclerosis

Usually ALS comes on slowly, starting out as weakness in one or more muscles. Only one leg or arm may be affected. People notice that they stumble, having trouble lifting things, or have trouble with using their hands. As the disease progresses, the person with ALS will not be able to stand or walk, have trouble moving around, and trouble talking and swallowing.

Diagnostic Tests

Although no diagnostic tests are specific to this disease, the following tests may aid in its diagnosis.

Electromyography may show abnormalities of electrical activity of involved muscles. Nerve conduction studies are usually normal.

X-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

Magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radio frequencies, and a computer to produce detailed images of organs and structures within the body.

Treatment

ALS has no cure. Treatment, which is supportive and based on the patient's symptoms, may include diazepam, dantrolene, or baclofen for spasticity and quinidine for relief of painful muscle cramps that occur in some patients. I.V. or intrathecal administration of thyrotropin-releasing hormone temporarily improves motor function in some patients but has no long-term benefits. Klonopin or inderal may be given for cerebellar dysfunction. Rehabilitative measures can help patients function effectively for a longer period, and mechanical ventilation can help them survive longer.

Prevention Tips

Genetic counseling may be advised if there is a family history of ALS. 



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