Encyclopedia Home / C / Charcot-Marie-Tooth disease

Charcot-Marie-Tooth disease

  • Definition

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


    Alternative Names

    Progressive neuropathic (peroneal) muscular atrophy; Hereditary peroneal nerve dysfunction; Neuropathy - peroneal (hereditary); Hereditary motor and sensory neuropathy


    Causes, incidence, and 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.