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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.

Causes

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:

• a slow-acting virus
• nutritional deficiency related to a disturbance in enzyme metabolism
• metabolic interference in nucleic acid production by the nerve fibers
• autoimmune disorders that affect immune complexes in the renal glomerulus and basement membrane.

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.

Diagnosis

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.

Treatment

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.

Special considerations

• Care begins with a complete neurologic assessment - a baseline for future evaluations of progressing disease.
• Implement a rehabilitation program designed to maintain independence as long as possible.
• Help the patient obtain equipment, such as a walker and a wheelchair. Arrange for a visiting nurse to monitor the patient's status, to provide support, and to teach the family about the illness.
• Depending on the patient's muscular capacity, assist with bathing, personal hygiene, and transfers from wheelchair to bed. Help establish a regular bowel and bladder routine.
• To help the patient handle increased accumulation of secretions and dysphagia, teach him to suction himself. He should have a suctioning machine handy at home to reduce his fear of choking.

Prognosis

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.