Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig disease, is the most common of the motor neuron diseases that cause muscle atrophy. (See Motor neuron disease.) Other motor neuron diseases include progressive muscular atrophy and progressive bulbar palsy. Symptoms don’t develop until age 50. A chronic, progressively debilitating disease, ALS may be fatal in less than 1 year or continue for 10 years or more, depending on the muscles it affects.
ALS affects about 1 of 100,000 people. The exact cause of ALS is unknown, but about 10% of cases have a genetic component. In these patients, it’s an autosomal dominant trait and affects men and women equally.
Other than a family member affected with the hereditary form, there are no known risk factors.
Progressive loss of muscle strength and coordination eventually interfere with everyday activities. Patients with ALS develop fasciculations, accompanied by atrophy and weakness, especially in the muscles of the feet and the hands. Other signs include impaired speech; difficulty chewing, swallowing, and breathing and, occasionally, choking and excessive drooling. Mental deterioration doesn’t occur, but patients may become depressed as a reaction to the disease.
Characteristic clinical features indicate a combination of upper and lower motor neuron involvement without sensory impairment. Electromyography and muscle biopsy indicate that the motor nerves aren’t functioning, yet sensory nerves are normal. Computed tomography scan and magnetic resonance imaging may help rule out other conditions, such as multiple sclerosis, spinal cord neoplasm, central nervous system syphilis, polyarteritis, syringomyelia, myasthenia gravis, progressive muscular dystrophy, and progressive strokes.
Management aims to control symptoms and provide emotional, psychological, and physical support. Riluzole may increase quality of life and survival but doesn’t reverse or stop disease progression.
Baclofen or diazepam help control spasticity that interferes with activities of daily living. Trihexyphenidyl or amitriptyline may be used for impaired ability to swallow saliva. Gastrostomy may be needed early to prevent choking; referral to an otolaryngologist is advised. Physical therapy, rehabilitation, and use of appliances or orthopedic intervention may be required to maximize function. Devices to assist in breathing at night or mechanical ventilation should be discussed, but the patient’s wishes should be respected.
Because mental status remains intact while progressive physical degeneration takes place, the patient acutely perceives every change. This threatens the patient’s relationships, career, income, muscle coordination, sexuality, and energy.
❑Implement a rehabilitation program designed to maintain independence as long as possible.
❑Help the patient obtain assistive equipment, such as a walker and a wheelchair. Arrange for a visiting nurse to oversee 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 suction machine handy at home to reduce the fear of choking.
❑To prevent skin breakdown, provide good skin care when the patient is bedridden. Turn him often, keep his skin clean and dry, and use pressure-reducing devices such as alternating air mattress.
❑If the patient has trouble swallowing, give him soft, solid foods and position him upright during meals. Gastrostomy and nasogastric tube feedings may be necessary if he can no longer swallow. Teach the patient (if he’s still able to feed himself) or his family how to administer gastrostomy feedings.
❑Provide emotional support. A discussion of directives regarding health care decisions should be instituted before the patient becomes unable to communicate his wishes. Prepare the patient and his family members for his eventual death, and encourage the start of the grieving process. Patients with ALS may benefit from a hospice program or the local ALS support group chapter.
Review other book chapters online related to Amyotrophic lateral sclerosis:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Professional Guide to Diseases (Eighth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2005 ISBN: 1-58255-370-X 
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