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Hereditary liability to pressure palsies

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Description

Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent acute sensory and motor neuropathy in a single or multiple nerves. The most common initial manifestation is the acute onset of a non-painful focal sensory and motor neuropathy in a single nerve (mononeuropathy). The first attack usually occurs in the second or third decade but earlier onset is possible. Neuropathic pain is increasingly recognized as a common manifestation. Recovery from acute neuropathy is usually complete; when recovery is not complete, the resulting disability is mild. Some affected individuals also demonstrate a mild-to-moderate peripheral neuropathy.

GeneReviews

  • Mode of Inheritance

  • Autosomal dominant inheritance

VARIANTS

52

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Genes

External Links

  • OMIM

    162500

  • Orphanet

    640

  • HPO
  • Medgen

    C0393814

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