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  August 27, 2008, 10:17 pm

What is Charcot-Marie-Tooth disease?

Charcot-Marie-Tooth is a family disease. This inherited neurolomuscular syndrome was discovered by three European doctors, Jean Charcot, Pierre Marie and Howard Tooth in the late eighteen hundreds. Their surnames are represented in the disease name, and this often misleads and confuses the general public because it does not reflect or explain the nature of the disease itself. CMT is considered to be the most commonly inherited form of peripheral neuropathy affecting approximately one in 2,500 adults.

CMT is diagnosed by clinical features of muscle atrophy, age of onset, electromyography (EMG), nerve biopsy and through genetic testing.

Charcot-Marie-Tooth disease (CMT) is a hereditary motor-sensory neuropathy (HMSN). HMSNs are a group of progressive neurological disorders that affect the motor and sensory units of the peripheral nervous system. CMT causes weakness because the nerve signals are impaired (demyelinated) before they reach the muscle. Over time this causes atrophy (a loss of muscle strength) in various areas of the body such as the feet, legs, hands, and diaphragm.

Although considered a "family disease", people are commonly affected differently. To date there is no known cure or treatment to arrest levels or rates of progression.

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