ALS Center of Oregon

 

What is ALS?

Amyotrophic lateral sclerosis (ALS) is also known as Lou Gehrig's disease. It is a progressive neurodegenerative disease. In healthy people, cells in the spinal cord and brain called ‘motor neurons’ control voluntary muscles. In ALS, these motor neurons deteriorate and the strength of the nerve signal that reaches the muscles decreases. Electrical signals from the nerves normally maintain muscle mass, and as these signals decrease, affected muscles shrink and weaken.

Symptoms

The initial and primary symptom is sudden and apparent muscle weakness. If weakness occurs first in the arms or legs, it is called ‘limb-onset.’ If the weakness first manifests as difficulty swallowing or speaking, it is called ‘bulbar-onset.’ Gradually, the weakness spreads to other regions of the body and results in paralysis.

Other symptoms include muscle stiffness, twitching, cramps and exaggerated reflexes. Pain is not specifically a symptom of ALS, but can occur as a result of muscle cramps, loss of muscle strength, and loss of mobility. Involuntary movements, such as eye twitches, bladder and bowel control, and sexual function are not usually affected, nor are touch, vision and hearing. ALS does not affect intelligence or reasoning.

Cause

The cause of ALS is unknown, and research has not been able to link it to environmental or age-related factors. In some cases, genetics may be involved; researchers have found at least one defective gene that might predispose people to ALS, though our understanding of how this gene operates is unclear.

ALS is not necessarily a genetic disease, however. Only 5-10% of the ALS population have a relative with ALS. Most people have ‘sporadic ALS,’ meaning that it occurs randomly. Approximately one out of 10,000 Americans has ALS, and ALS is not contagious.

Diagnosis

ALS is usually difficult to diagnose and an experienced physician needs to perform a variety of tests and exams. Usually a thorough medical history is taken, followed by a neurological exam and an EMG (an electromyogram) to measure the electrical signals reaching the muscles. Sometimes physicians look at blood tests, MRI and CT scans, and muscle biopsies to rule out other diagnoses. Although the criteria for the diagnosis of ALS are specific, the diagnosis is not based on one test.

Treatment

There is no cure for ALS. At present, only the drug Rilutek is known to slow the progression of ALS, and prolongs survival by about two months. Research is currently underway into new treatments using the medicines minocycline, dronabinol, and ceftriaxone, and new trials are continually proposed.

Most treatments are directed towards relieving symptoms such as muscle twitching, cramps and excessive salivation that can occur as a result of ALS. Drugs, physical therapy, occupational therapy, swallowing/speech therapy, massage, and respiratory therapy are all used in the care of ALS. Ventilators and feeding tubes are used to support life in advanced ALS.

Prognosis

The progression of ALS is very individual. Each person's disease is different, and each person makes different choices in his or her treatment plan. We encourage patients and their families to plan for the future by being informed, taking advantage of treatment options and by completing Advanced Directives.

 

 

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ALS Center of Oregon
Oregon Health & Science University
email ALS@ohsu.edu
Website comments or suggestions can be directed to
Neuromuscular Webmaster

phone: (503) 494-5236 fax: (503) 494-0966

Page Updated:Thursday, October 05, 2006 11:58:02 AM -0700


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