Functional involvement of central nervous system in mitochondrial disorders. Academic Article uri icon

Overview

abstract

  • Thirty-nine patients with mitochondrial diseases were studied with somatosensory and motor evoked potentials. Sixteen patients (41%) had clinical and 12 (31%) had neuroradiological evidence of central nervous system involvement. The overall incidence of electrophysiological abnormalities was 64%. Abnormal evoked potentials were also found in a significant percentage (33%) of patients with pure myopathic forms of mitochondrial diseases and in an asymptomatic carrier of MERRF mutation. Of the individual tests, somatosensory evoked potentials were abnormal in 49% of the patients and motor evoked potentials were abnormal in 46% of the patients. The outcome is that electrophysiological evidence of central nervous system involvement is present in a high percentage of patients with mitochondrial disorders, and that the threshold for central nervous system electrophysiological abnormalities is well below that for clinical and/or radiological manifestations.

publication date

  • June 1, 1997

Research

keywords

  • Central Nervous System
  • Evoked Potentials, Motor
  • Evoked Potentials, Somatosensory
  • Mitochondrial Myopathies

Identity

Scopus Document Identifier

  • 0030993609

Digital Object Identifier (DOI)

  • 10.1016/s0924-980x(97)96671-6

PubMed ID

  • 9216485

Additional Document Info

volume

  • 105

issue

  • 3