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Hereditary neuropathy with pressure palsies (HNPP)

Overview

HNPP is a neurological disorder, which is autosomal dominant inherited, with an increased tendency for nerve compression at common compression sites like elbow and fibular head.

Caused due to mutation in PMP22 gene

Inheriting the mutated PMP22 gene from a parent

  • Weakness such as wrist drop/ foot drop/ weakness of small muscles of hand after prolonged posturing
  • Tingling/ numbness/ pain
  • Atrophy of muscles
  • Loss of sensation
  • The weakness may persist for a few weeks or may become permanent

  • Nerve conduction studies for site and severity of nerve compression
  • MR neurography
  • Nerve biopsy (rarely required)
  • Genetic testing

Neurologist

  • Short course of steroids may be warranted
  • Orthosis and assistive devices
  • Physiotherapy
  • Avoidance of compression at common sites prone for nerve injury

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