Charcot Marie Tooth Disease
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History/Causes
   Charcot-Marie-Tooth Disease was first described and named after three physicians in 1886.  Jean-Martin Charcot was a famous French neurologist who has been called the Father of Neurology.  Pierre Marie was a pupil of Charcot�s and together they published their findings in February of 1886 in France.  Howard Henry Tooth, at the same time, was working on his thesis, titled The Peroneal Type of Progressive Muscular Atrophy, for an MD degree at Cambridge University in England.  The disease was called Charcot-Marie for awhile until Tooth was added because of his concurrent efforts and assistance.(2,4)  
    Charcot-Marie-Tooth is abbreviated by CMT.  It is one of the most common inherited disorders of the nervous system and affects on average 115,000 Americans.  It is not considered a single disease but a collection of diseases caused by defects in different genes.  This is referred to as a syndrome.  CMT is not life-threatening and rarely ever affects the brain.  It causes damage to peripheral nerves which connect the brain and spinal cord to muscles and organs.  There is no cure for this syndrome but there are treatments that can help to manage the symptoms.  CMT is not contagious and the gene mutations are usually inherited.(1-3,5)
    CMT is caused by defects in genes which are recipes for making the proteins that serve essential functions in our bodies.  Each type of CMT is linked to a specific gene.  The proteins that those genes make are found within peripheral nerves and involved in the function and structure of the myelin sheath or peripheral nerve axon.  Peripheral nerves are made up of fibers called axons, which are surrounded by myelin made by Schwann cells.  Myelin insulates axons to allow for a faster conduction speed of electrical signals.  The slow degeneration of these nerves loses the capability to communicate with distant targets in the limbs.  The longest axons in the body are the most sensitive to damage.  This allows for an explanation of why CMT mostly causes motor and sensory problems in the extremities.  Degeneration of motor nerves produces muscle weakness and wasting in the extremities.  Degeneration of sensory nerves produces a decreased ability to feel heat, pain, and cold.(1, 3-5) 













References
1Charcot-Marie-Tooth Disorder Fact Sheet.  National Institute of Neurological Disorders and Stroke.  Available at:  http://www.ninds.nih.gov/disorders/charcot_marie_tooth/detail_charcot_marie_tooth.htm.  Accessed February 15, 2005.

2CMT History.  CMTnet page.  Available at:  http://users.rcn.com/smith.ma.ultranet/CMTneto.html.  Accessed February 15, 2005. 

3Facts About Charcot-Marie-Tooth Disease and Dejerine-Sottas Disease.  MDA Publications page.  Available at:  http://www.mdausa.org/publications/fa-cmt.html#types.  Accessed February 15, 2005. 

4Stimson D.  From Clear-Cut Endings to Complex Beginnings: Researchers Probe the Origins of Charcot-Marie-Tooth Disease.  Quest.  2001; 8 (1). 

5What is CMT.  CMTnet page.  Available at:  http://users.rcn.com/smith.ma.ultranet/CMTneto.html.  Accessed February 15, 2005.
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