Charcot-Marie-Tooth disease : Overview, Causes, & Risk Factors

Alternate Names : Progressive neuropathic (peroneal) muscular atrophy, Hereditary peroneal nerve dysfunction, Neuropathy – peroneal (hereditary), Hereditary motor and sensory neuropathy

Definition

Charcot-Marie-Tooth disease is a group of inherited disorders that affect the peripheral nerves (those outside the brain and spine).

Overview, Causes, & Risk Factors

Charcot-Marie-Tooth is one of the most common inherited nerve-related disorders. Defects in at least 14 genes cause different forms of this disease.

The disease involves damage to the covering (myelin sheath) around nerve fibers. In some, the disease causes destruction of the myelin sheath. In others, the central (axon) portion of the nerve cell wears away.

Nerves that stimulate movement (the motor nerves) are most severely affected. The nerves in the legs are affected first and most severely.

Pictures & Images

Central nervous system and peripheral nervous system

The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.


Review Date : 8/26/2009
Reviewed By : Chad Haldeman-Englert, MD, Wake Forest University School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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