Functional involvement of central nervous system in mitochondrial disorders

V. Di Lazzaro, D. Restuccia, S. Servidei, M. Valeriani, R. Nardone, G. Manfredi, G. Silvestri, E. Ricci, P. Tonali

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)171-180
Number of pages10
JournalElectroencephalography and Clinical Neurophysiology - Electromyography and Motor Control
Issue number3
Publication statusPublished - Jun 1997


  • Mitochondrial myopathies
  • Motor evoked potentials
  • Somatosensory evoked potentials

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)


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