Amyotrophic Lateral Sclerosis (ALS)
Commonly called Lou Gehrig's disease, after the New York Yankee first baseman who died of this disorder, amyotrophic lateral sclerosis (ALS) is the most common of the motor neuron diseases 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.
More than 30,000 Americans have ALS; about 5,000 new cases are diagnosed each year, with men affected three times more often than women. The exact cause of ALS is unknown, but about 5% to 10% of ALS cases have a genetic component. In these cases, it's an autosomal dominant trait and 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.
Signs and symptoms
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 reaction to the disease. Progressive bulbar palsy may cause crying spells or inappropriate laughter.
Characteristic clinical 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. Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm. Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation . Pharmacists can give advice on the proper use of medications and monitor a patient's prescriptions to avoid risks of drug interactions.
A dietitian can advise you on foods that are nutritious but easy to swallow. As the disease progresses and you have greater difficulty swallowing, you may consider a feeding tube inserted into your stomach to receive nutrition and avoid choking.
ALS usually progresses rapidly, and leads to death from respiratory infection within three to five years in most cases. The slowest disease progression is seen in those who are young and have their first symptoms in the limbs. About 10% of people with ALS live longer than eight years.
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