Lou Gehrig's disease
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amyotrophic lateral sclerosis
The disease presents in adulthood, usually between the ages of 40 and 70, and affects men two to three times more often than women. The initial symptom is weakness of skeletal muscles, especially in the limb. As the disease progresses the patient has difficulty swallowing and talking, with dyspnea as the accessory muscles of respiration are affected. Eventually muscles atrophy and the patient becomes a functional quadriplegic. Mentation is not affected, so that the patient remains alert and aware of functional loss and the inevitable outcome. Although there may be periods of remission, the disease usually progresses rapidly, with death in 2 to 5 years. The cause of ALS is not known and there is no cure. Treatment is intended to provide symptomatic relief, prevent complications, and maintain optimal function as long as possible.
Intervention is planned and implemented according to each patient's needs at specific times during the course of the illness. In general, the major problems encountered are those related to (1) dysphagia and the need to meet nutritional requirements and avoid aspiration, (2) dyspnea and maintenance of blood gases within normal range, (3) aphasia and impaired verbal communication, (4) weakness, impaired mobility, and activity intolerance, (5) constipation, (6) pain and discomfort due to muscle cramps, and (7) alteration in self-concept and body image.
The patient and family also will need assistance in managing home care, coping with the effects of the illness, and maintaining optimal functioning in the patient. Community health nurses and home health care professionals and paraprofessionals should be available to provide a variety of services including physical therapy, occupational therapy, social services, mental health care, and medical and nursing care.
A resource agency that can provide assistance and information to ALS patients and their families is the Amyotrophic Lateral Sclerosis Association, 21021 Ventura Blvd., Suite 321, Woodland Hills, CA 91364-2206, (800) 782–4747; http://www.alsa.org.
Lou Gehrig's disease(lo͞o′ gĕr′ĭgz)
amyotrophic lateral sclerosisA chronic, progressive, degenerative, motor neurone disease, characterised by upper limb weakness, atrophy and focal neurologic signs.
Epidemiology Incidence 0.5–1.5/105, more common in men, usually >age 50; occurs randomly throughout the world with local clustering on the Kii Peninsula, Japan, and Guam, where it is associated with dementia, parkinsonism, and Alzheimer’s disease.
Clinical Loss of fine motor skills, triad of atrophic weakness of hands and forearms; leg spasticity; generalised hyperreflexia.
Management Possibly riluzole.
Terminal care Where allowed, one-fifth of all ALS patients choose to die by euthanasia.
Lou Gehrig's diseaseA popular term in the USA for AMYOTROPHIC LATERAL SCLEROSIS. Lou Gherig (1904–41) was a baseball player for the Yankees of countrywide fame and popularity who, to the distress of all who knew him, began to develop signs of ALS in 1938.
Patient discussion about Lou Gehrig's disease
Q. What are the presenting signs of ALS? Are the upper or lower extremeties affected initialilly?
Other presenting signs may be problems with speaking or swallowing, although these are less common.
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