Brachial plexus involvement as the only expression of hereditary neuropathy with liability to pressure palsies

Muscle Nerve. 2001 Aug;24(8):1093-6. doi: 10.1002/mus.1116.

Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant focal neuropathy characterized by recurrent entrapment neuropathies. Single members of families with HNPP and brachial plexus involvement have been reported previously. We describe a family with three affected members and in which the only symptom of entrapment was recurrent brachial plexopathy. The diagnosis of HNPP in this family was confirmed by both neurophysiological methods and DNA analysis. The distinction between HNPP and hereditary neuralgic amyotrophy is discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brachial Plexus Neuropathies / complications
  • Brachial Plexus Neuropathies / diagnosis*
  • Brachial Plexus Neuropathies / physiopathology*
  • Chromosomes, Human, Pair 17 / genetics
  • DNA Mutational Analysis
  • Electrodiagnosis
  • Electromyography
  • Genes, Dominant
  • Genetic Predisposition to Disease
  • Humans
  • Loss of Heterozygosity
  • Male
  • Median Nerve / physiopathology
  • Middle Aged
  • Myelin Proteins / deficiency
  • Myelin Proteins / genetics
  • Nerve Compression Syndromes / complications
  • Nerve Compression Syndromes / diagnosis*
  • Nerve Compression Syndromes / physiopathology*
  • Neural Conduction
  • Pedigree
  • Peroneal Nerve / physiopathology

Substances

  • Myelin Proteins
  • PMP22 protein, human