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Alternative names Return to top
Lou Gehrig's disease; ALSDefinition Return to top
A disease of the motor nerve cells in the brain and spinal cord, causing progressive loss of motor control.Causes, incidence, and risk factors Return to top
Amyotrophic Lateral Sclerosis is caused by progressive loss of motor nerves in the spinal cord and brain. In about 10% of cases, ALS is caused by a genetic defect. In other cases, the cause of the nerve deterioration is unknown.
ALS affects approximately 1 out of 100,000 people.
Except for having a family member affected with the hereditary form of the disease, there are no known risk factors.
Symptoms Return to top
Symptoms usually do not develop until until after age 50. Progressive loss of muscle strength and coordination eventually interfere with the ability to perform routine activities, such as going up steps, getting out of a chair, or swallowing. Occasionally, breathing or swallowing muscles may be the first to be affected.
As the disease progresses, more muscle groups are affected and patients become progressively incapacitated. There is no effect on the ability to think or reason.
Symptoms include:
Signs and tests Return to top
A neuromuscular examination indicates weakness, often beginning in one limb or in proximal groups (such as shoulders or hips). There may be muscle tremors, spasms, twitching, or muscle atrophy (loss of tissue). Atrophy and twitching of the tongue are common.
The gait may be stiff or clumsy. Reflexes may be abnormal, including loss of the gag reflex. Some patients have "emotional incontinence" in which it is hard to control crying or laughing.
Treatment Return to top
There is no known cure for ALS. Riluzole may prolong life, but does not reverse or stop disease progression.
Treatment is aimed at controlling the symptoms. Baclofen or diazepam may be used to control spasticity that interferes with activities of daily living. Trihexyphenidyl or amitriptyline may be prescribed for people with impaired ability to swallow saliva.
Physical therapy, rehabilitation, use of appliances (such as braces or a wheelchair) or orthopedic intervention may be required to maximize muscle function and general health.
Choking is common and there may be an early need for placement of a tube into the stomach for feeding (gastrostomy). A referral to an otolaryngologist may be advised. A nutritionist is helpful to prevent weight loss, especially for patients with limitied ability to swallow.
The use of devices to assist in breathing includes machines that are only used at night as well as mechanical ventilation. Patients should discuss their wishes regarding artificial ventilation with their doctors.
Support Groups Return to top
Emotional support is vital in coping with the disorder, because mental functioning is not affected. Groups such as the ALS Association may be available to assist in coping with the disorder. See ALS - support group.
Expectations (prognosis) Return to top
There is progressive loss of ability to function or care for oneself. Death often occurs within 3 to 5 years of diagnosis, although there are some rare cases of patients living decades.
Complications Return to top
Calling your health care provider Return to top
Call your health care provider if symptoms suggest ALS, particularly if there is a family history of the disorder.Prevention Return to top
Genetic counseling may be advised if there is a family history of ALS. Update Date: 11/3/2002 Updated by: Joseph V. Campellone, MD; Division of Neurology, Cooper Hospital/University Medical Center, Camden, NJ. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 September 2004 |