Amyotrophic lateral sclerosis

Basic Information

Description

Amyotrophic lateral sclerosis (ALS) is a condition in which nerves that control the body's muscle activity deteriorate (degenerate). ALS affects the nerves (motor neurons) located in the brain (medulla and cortex) and the spinal cord. The result is progressive weakness and wasting (atrophy) of the muscles. Lou Gehrig was a well-known athlete whose baseball career and life were prematurely ended as a result of this disease. Hence, it is often referred to as Lou Gehrig's Disease. The cause of ALS is not known. Some theoretical causes are genetic or inherited factors, environmental poisons (toxins), viral infections, or dysfunction of the body's defense system (immunologic factors).

One or two cases per 100,000 people are diagnosed each year in the US. Usually affecting people over forty years of age, it is more common in men than women. About ten percent of ALS cases run in families (familial).

Symptoms may be confused with neurologic complications of Lyme disease.

Frequent signs and symptoms

• Muscle twitching and weakness, beginning in the hands and spreading to the arms and legs. Weakness eventually affects muscles that control breathing and swallowing.
• Muscle cramps.
• Stiffening and spasticity of muscle groups.
• Unexplained weight loss.
• Slurring of speech.
• Mental deterioration usually does not occur.
• Sudden involuntary bursts of laughter or crying.

Causes

Unknown.

Risk increases with

• Age over 40.
• Family history of ALS.
• Incidence is highest among those whose occupation demands strenuous physical labor.

Preventive measures

Cannot be prevented at present.

Expected outcomes

Death usually occurs within two to seven years from the onset of symptoms. However, exceptions do occur. Some people have lived more than twenty years after diagnosis.

Possible complications

As the disease progresses, the individual becomes unable to speak, swallow, or move. The muscles used in respiration usually become affected making breathing difficult. Resulting complications may include choking, pressure sores, and respiratory complications. Swallowing dysfunction may cause pneumonia.

Treatment of Amyotrophic Lateral Sclerosis (ALS)

General measures

• Diagnostic testing will include a electromyography to measure nerve conduction.
• There is no specific treatment. Supportive care is provided to control symptoms and for complicating emergencies.
• Obtain good nursing care to prevent pressure sores.
• Learn to do self-suction in order to handle increased accumulation of secretions in the lungs.
• Psychotherapy or counseling to learn to cope with disability.
• Eventual hospitalization or nursing-home care.
• Patients may benefit from a hospice program or local chapter of the ALS support group.

Medications

• There are no medications to treat ALS.
• Antibiotics to fight infection if pneumonia develops.
• Baclofen may help reduce spasticity.
• Antidepressant may help to decrease saliva production.

Information	Brand	Generic	Label	Rating
	Elavil	Amitriptyline
	Neurontin	Gabapentin
	Lioresal	Baclofen

Valium (Diazepam)

Activity

• Stay as active as possible. Weakness will gradually limit capability. A rehabilitation program can help in maintaining independence as long as possible.
• Obtain equipment that will aid in mobility, such as walker or wheelchair.

Diet

• If swallowing is difficult, soft, easy-to-swallow foods.
• May require tube feedings eventually.

Appropriate specialists

Neurologist, physical therapist and occupational therapist.

Last updated 16 December 2011