Also Known As
* ALS or Lou Gehrig's disease
Description
* Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, is a disorder that causes the loss of control of the voluntary muscles due to a degeneration of the nerve cells in the brain and spinal cord. It only affects the anterior horn cells (motor neurons of the brain and the spinal cord) and does not involve the sensory nerve cells.
Symptoms
* Slowly progressive muscle weakness of the extremities, muscle stiffness and fatigue
* Difficulty in swallowing, chewing, breathing and speaking due to muscle weakness
* Weight loss due to muscle waste
* Fasciculations (muscle twitching)
* Muscle Cramps
Cause
* The cause is unknown. About 5 to 10 % of all ALS cases are inherited, while others result from the mutation of specific gene.
* ALS generally begins between the ages of 30 and 60.
How The Diagnosis Is made
* A diagnosis is made by patient history and a neurological examination. Patients do not show physical signs of sensory dysfunction. All symptoms are related with a muscle weakness of each part of the body. An Electromyography is helpful in diagnosing the damage of motor neurons, while brain MRIs and EMGs help to rule out other diseases that can cause progressive motor weakness.
Treatment
* There is no known cure for ALS
* Rriluzole (RILUTEK), approved by the FDA, may reduce the mortality and prolong the survival of ALS patients.
* Treatments are largely supportive, and are aimed at the control of symptoms and complications, such as muscle stiffness, swallowing difficulty and difficulty with breathing.
* Physical therapy and use of special equipment (such as braces or a wheelchair) may enhance a feeling of independence, as well as insuring the safety of the patient.
Complications
* Aspiration of food or fluid
* Pneumonia
* Respiratory failure
Prognosis
* ALS is a progressive disease without remissions and relapses. Most people die within 3 to 5 years from the onset of symptoms. However, about 20 percent of ALS patients survive for 5 or more years.
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