Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, is a progressive neurological disease affecting 30,000 Americans with about 5,000 new cases occurring in the United States each year.
ALS belongs to a class of disorders known as motor neuron diseases. Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. The loss of these cells causes the muscles under their control to weaken and waste away, leading to paralysis. It is usually fatal within five years of diagnosis.
ALS manifests itself in different ways, depending on which muscles weaken first. Symptoms may include tripping and falling, loss of control in hands and arms, difficulty speaking, swallowing and/or breathing, persistent fatigue, and twitching and cramping. ALS strikes in mid-life. Men are about one-and-a-half times more likely to have the disease as women.
Because ALS affects only motor neurons, the disease does not impair a person's mind, personality, intelligence, or memory. It does not affect a person's ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions.
There is no cure for ALS, nor is there a proven therapy that will prevent or reverse its course. The Food and Drug Administration (FDA) recently approved riluzole, the first drug that has been shown to prolong the survival of ALS patients. Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials showed that riluzole prolongs survival by several months, mainly in those with difficulty swallowing. The drug also extends the time before a person needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects.
Physical therapy and special equipment can enhance independence and safety throughout the course of ALS. Low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression. Range of motion and stretching exercises can help prevent painful spasticity and muscle contractures. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile.
When the muscles that assist in breathing weaken, use of ventilatory assistance (intermittent positive pressure ventilation [IPPV] or bilevel positive airway pressure [BIPAP]) may be used to aid breathing during sleep. When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time.
Social workers and home care and hospice nurses help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease. Social workers provide support such as assistance in obtaining financial aid, arranging durable power of attorney, preparing a living will, and finding support groups for patients and caregivers.
National Institute on Neurological Disorders and Stroke, ALS Association