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Hereditary Neuropathies

By Michael Rubin, MDCM, Professor of Clinical Neurology;Director, Neuromuscular Service and EMG Laboratory, Weill Cornell Medical College;New York Presbyterian Hospital-Cornell Medical Center

Hereditary neuropathies affect the peripheral nerves, causing subtle symptoms that worsen gradually.

Hereditary neuropathies may affect only motor nerves (motor neuropathies), sensory and autonomic nerves (sensory neuropathies), or sensory and motor nerves (sensory and motor neuropathies). Motor nerves control muscle movement, and sensory nerves carry sensory information—about such things as pain, temperature, and vibration—to the brain. Autonomic nerves regulate involuntary body processes.

Some hereditary neuropathies are relatively common but often are not recognized.

The genes responsible for many hereditary neuropathies have been identified. These neuropathies include

When the sensory and autonomic nerves are affected, the ability to feel pain and changes in temperature is impaired more than the ability to sense vibration and position (knowing where the arms and legs are). The hands and feet are affected most. Because people cannot feel pain, they may injure their feet and not know it. Such injuries increase the risk of infections, including bone infections, and of joint damage (called neurogenic arthropathy, or Charcot joints—see Neurogenic Arthropathy).

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