Commonly called Lou Gehrig disease, after the New York Yankee first baseman who died of this disorder, amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease causing muscular atrophy. Other motor neuron diseases include progressive muscular atrophy and progressive bulbar palsy. Onset occurs between ages 40 and 70. A chronic, progressively debilitating disease, ALS is rapidly fatal.
The disease is progressive, with death resulting from respiratory paralysis (the median survival is 3 to 5 years), and it’s three times more common in men than in women. The exact cause of ALS is unknown, but 5% to 10% of ALS cases have a genetic component. In these cases, it’s an autosomal dominant trait that affects men and women equally.
ALS and other motor neuron diseases may result from:
Precipitating factors for acute deterioration include trauma, viral infections, and physical exhaustion.
Patients with ALS develop fasciculations, accompanied by atrophy and weakness, especially in the muscles of the forearms and the hands. Other signs include impaired speech; difficulty chewing, swallowing, and breathing, particularly if the brain stem is affected; and, occasionally, choking and excessive drooling.
Mental deterioration doesn’t usually occur, but patients may become depressed as a result of the disease. Progressive bulbar palsy may cause crying spells or inappropriate laughter.
Characteristic features indicate a combination of upper and lower motor neuron involvement without sensory impairment. Electromyography and a muscle biopsy help show nerve, rather than muscle, disease. The protein content of cerebrospinal fluid is increased in one-third of patients, but this finding alone doesn’t confirm ALS.
Diagnosis must rule out multiple sclerosis, spinal cord neoplasm, polyarteritis, syringomyelia, myasthenia gravis, and progressive muscular dystrophy.
Management aims to control symptoms and provide emotional, psychological, and physical support.
CLINICAL TIP: A new drug, riluzole, may provide a treatment for ALS that increases survival time and quality of life.
Review other book chapters online related to Amyotrophic lateral sclerosis:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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Title: Handbook of Diseases Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2003 ISBN: 1-58255-266-5 
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