Friday, October 24, 2008

 

Lou Gehrig’s Disease: Amytrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is best known for its most famous victim, Lou Gehrig. It is a devastating neurologic disease that eventually ends in the death of its victims.

ALS is one of a group of motor neuron diseases that results from degeneration of motor nerve cells at their origin in the spinal cord and peripheral extensions. Motor nerves stimulate and nourish voluntary muscles. When they cease to function, muscles become wasted and eventually paralyzed. Patients suffering from ALS lose their ability to move including eating, breathing and speaking.

ALS affects approximately 30,000 Americans and 60 percent are male. Life expectancy is between three and 10 years. Respiratory complications are most often the cause of death. Some patients choose to live longer through the use of artificial nutrition and a respirator. ALS does not affect a person’s ability to think. The loss of speech is the greatest obstacle and significantly diminishes a patient’s quality of life.

Although there is an inherited form of ALS, the overwhelming majority of patients, more than 90 percent, suffer from the sporadic, non-inherited form.

The diagnosis of ALS is made by the combination of a thorough neurological examination and diagnostic studies that include nerve conduction studies and electromyography. There is no specific marker in blood or tissue samples.

Many treatments have been attempted over the years. The only FDA- approved drug at this time, Riluzole, marginally slows disease progression, extending survival an average of three months. Rehabilitation, including the use of mobility aids and devices for communication, are most effective.

Little progress has been made in finding a cure for this devastating disease. Like most medical challenges, improved funding may have a remarkable impact.

On Nov. 2, a benefit walk for ALS will be held at Norwich Free Academy. The proceeds will benefit the ALS Association. If you are interested in supporting this event, contact musiqueman65@comcast.net or visit norwichfreeacademy.com.

Anthony G. Alessi, MD, is Chief of Neurology at The William W. Backus Hospital and in private practice at NeuroDiagnostics, LLC in Norwich. To contact Dr. Alessi, email him aalessi@wwbh.org. To purchase his recently published “Healthy Sports: A Doctor’s Lessons for a Winning Lifestyle” book, visit backushospital.org/alessi.

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