Stephen J. Schueler, M.D.

Overview Symptoms

Dejerine-Sottas Disease Overview

Another name for Dejerine-Sottas Disease is Charcot-Marie-Tooth Disease.

What is Charcot-Marie-Tooth disease?
Charcot-Marie-Tooth disease is a group of inherited disorders that affect the peripheral nerves. The peripheral nerves are the nerves located outside the brain and spine. Any disease process that disturbs the function of these nerves is referred to as a peripheral neuropathy. There are many forms of Charcot-Marie-Tooth disease, but all cause some kind of abnormality in the myelin sheath. Myelin is a substance that coats nerves fibers, allowing them to properly transmit electrical impulses.

What are the symptoms of Charcot-Marie-Tooth disease?
The symptoms of Charcot-Marie-Tooth disease normally occur between mid childhood and early adulthood and include foot drop, very high arched feet, numbness in the foot or leg, weakness of the hips, legs or feet, and lower leg muscle loss. Symptoms usually worsen very slowly over time. Later symptoms of Charcot-Marie-Tooth disease can occur in the arms and hands and may include a claw like deformity in the hand. Muscle weakness and loss of balance can making walking difficult.

How does the doctor treat Charcot-Marie-Tooth disease?
Treatment for Charcot-Marie-Tooth disease may include braces, orthopedic shoes or orthopedic surgery to help a person walk easier. Physical and occupational therapy may also help in maintaining muscle strength. There is no known cure for Charcot-Marie-Tooth disease. Treatment involves managing complications of the disease. Pain medications may be required to control chronic pain that is often associated with this condition.

Continue to Dejerine-Sottas Disease Symptoms

Last Updated: Dec 23, 2010 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Dejerine-Sottas Disease References
  1. Banchs I, Casasnovas C, Alberti A, De Jorge L, Povedano M, Montero J, Martinez-Matos JA, Volpini V. J Biomed Biotechnol. 2009;2009:985415. Epub 2009 Oct 8. Review. [19826499]
  2. Bird TD. In: Pagon RA, Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-.1998 Sep 28 [updated 2009 Apr 16]. [20301532]
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