Amyotrophic lateral sclerosis (ALS) is a progressive nervous system disorder. It gradually destroys the nerves responsible for muscle movement. Over time, ALS leads to total paralysis of muscle movement, including respiration.
Prognosis is poor in most cases because of the progressive nature of the condition due to eventual respiratory failure. After diagnosis is made, lifespan ranges from 2-5 years. The 5 year survival rate is 25% and up to 10% of patients will survive more than 10 years. In general, the younger the age of onset, the slower the disease progresses.
The cause of ALS is not known, but it appears that genes may play a role in a very small number of cases. Also, research has shown that there may be a reduced response to cell stress, as well as cell toxicity, related to certain protein build-up in the brain.
A risk factor is something that increases your chance of getting a disease or condition. Risk factors include:
Symptoms include:
The doctor will ask about your symptoms and medical history, and perform a physical exam. There are no tests that definitively diagnose ALS, but tests may be used to rule out other medical conditions.
Tests may include:
There is currently no cure for ALS.
For you and your family, a multidisciplinary approach may work best. This approach may include:
Treatments options include:
The drug, called riluzole , has been approved for ALS with a clinical trial revealing a modest lengthening of survival. The drug may slightly improve functioning, but it doesn't stop the disease from progressing. A study, though, showed that the addition of lithium carbonate (a medication used to treat mood disorders) to riluzole may slow the progression of ALS and prolong survival. *¹ Other drugs are also being studied.
Your doctor may prescribe these medications for symptom
RESOURCES:
ALS Association
http://www.alsa.org/
National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov/
References:
Amyotrophic lateral sclerosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated February 4, 2009. Accessed February 23, 2008.
Bradley WG, Daroff RB. Neurology In Clinical Practice . Philadelphia, PA: Butterworth Heinemann Publishing; 2004.
Lou JS. Amyotrophic lateral sclerosis. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. Available at: http://www.medlink.com . Accessed February 4, 2009.
Goetz CG. Textbook of Clinical Neurology . Philadelphia, PA: WB Saunders Company; 1999.
Kasper DL, Braunwald E, Fauci AS, et al. Harrison's Principles of Internal Medicine . 16th ed. New York, NY: The McGraw-Hill Companies; 2005.
Miller RG, Mitchell JD, Lyon M, et al. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). The Cochrane Library . Issue 3. Chichester, UK: John Wiley & Sons, Ltd; 2005.
National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/ . Accessed October 12, 2005.
Samuels MA, Feske SK. Office Practice of Neurology . Philadelphia, PA: Churchill Livingstone; 2003.
Walling AD. Amyotrophic lateral sclerosis: Lou Gehrig's disease. Am Fam Physician . 1999;59:1489-1496.
*¹ 4/17/2008 DynaMed Systematic Literature Surveillance DynaMed's Systematic Literature Surveillance : Fornai F, Longone P, Cafaro L, et al. Lithium delays progression of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA. 2008;105:2052-2057. Epub 2008 Feb 4.
Last reviewed January 2009 by J. Thomas Megerian, MD, PhD, FAAP
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