The peroneal muscular atrophy syndrome. Clinical, genetic, electrophysiological and nerve biopsy studies part 2. Observations on pathological changes in sural nerve biopsies

R. Madrid, Walter G Bradley, C. J F Davis

Research output: Contribution to journalArticle

65 Scopus citations

Abstract

The light- and electron-microscopic, single teased nerve fibre and morphometric studies of a series of 17 sural nerve biopsies from patients with peroneal muscular atrophy are presented. The cases are divided into the following groups according to the criteria of Davis, Bradley and Madrid (1977): Hypertrophic Neuropathy Group; Intermediate Group; Neuronal Sensorimotor Group; Neuronal Motor Group. The Hypertrophic Neuropathy Group had nerve hypertrophy and marked segmental demyelination with onion bulb formation. The Intermediate Group also had segmental demyelination and onion bulb formation, but nerve hypertrophy was not seen, and axonal degeneration and regeneration were prominent. The Neuronal Sensorimotor Group cases were all sporadic, and showed some onion bulbs, paranodal demyelination and evidence of axonal degeneration and regeneration. The sensory nerve biopsy in the Neuronal Motor Group showed no major abnormality apart from some cluster formation indicating axonal regeneration. The data tend to support the classification of peroneal muscular atrophy proposed by Davis et al. (1977), though there was overlap between the groups in individual pathological parameters.

Original languageEnglish
Pages (from-to)91-122
Number of pages32
JournalJournal of the Neurological Sciences
Volume32
Issue number1
DOIs
StatePublished - Jan 1 1977
Externally publishedYes

    Fingerprint

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

Cite this