Merck Manual

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Hereditary Neuropathies

By

Michael Rubin

, MDCM, New York Presbyterian Hospital-Cornell Medical Center

Reviewed/Revised Apr 2022 | Modified Sep 2022
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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)

  • 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 Autonomic nervous system The peripheral nervous system consists of more than 100 billion nerve cells (neurons) that run throughout the body like strings, making connections with the brain, other parts of the body, and... read more regulate involuntary body process.

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

The genes responsible for many hereditary neuropathies and other hereditary disorders that cause neuropathies have been identified. The following are examples:

When the sensory nerves are affected, the ability to feel pain and changes in temperature may be 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 risk of joint damage (called neurogenic arthropathy Neurogenic Arthropathy Neurogenic arthropathy is caused by progressive joint destruction, often very rapid, that develops because people cannot sense pain, continually injure joints, and thus are not aware of the... read more , or Charcot joints). If the ability to sense vibration and position is affected, people have problems with balance and walking.

When motor nerves are affected, muscles weaken, waste away (atrophy), and can become completely paralyzed.

NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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