Is early onset cerebellar ataxia with retained tendon reflexes identifiable by electrophysiologic and histologic profile?. A comparison with Friedreich's ataxia

L. Santoro, A. Perretti, A. Filla, G. De Michele, B. Lanzillo, F. Barbieri, C. Crisci, P. Gasparo Rippa, G. Caruso

Research output: Contribution to journalArticlepeer-review

Abstract

An electrophysiologic and histologic study was performed on 18 patients affected by early onset cerebellar ataxia with retained tendon reflexes (EOCA). Sensory and motor conduction velocity (SCV, MCV) was measured along peripheral nerves in all patients, somatosensory (SSEP) and brainstem auditory evoked potentials (BAEP) were recorded in 13; cortical stimulation (CS) in 12, and sural nerve biopsy in 4 patients were also performed. The results as a whole allow a division of EOCA patients into 2 groups: with (7 patients) and without (11 patients) peripheral neuropathy. Among EOCA patients with neuropathy a differential diagnosis with Friedreich's disease patients was not possible according to BAEPs and CS, while SSEPs could differentiate 2 out 5 patients in whom they were performed.

Original languageEnglish
Pages (from-to)43-49
Number of pages7
JournalJournal of the Neurological Sciences
Volume113
Issue number1
DOIs
Publication statusPublished - 1992

Keywords

  • Early onset cerebellar ataxia
  • Evoked potential
  • Friedreich's ataxia
  • Nerve biopsy
  • Nerve conduction

ASJC Scopus subject areas

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

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