Evoked potentials in inherited ataxias: A multimodal electrophysiological study

B. Lanzillo, A. Perretti, L. Santoro, L. Pelosi, A. Filla, G. De Michele, G. Caruso

Research output: Contribution to journalArticlepeer-review


A multimodal electrophysiological study, including median nerve somatosensory evoked potentials (SSEPs), motor cortical stimulation (CS) and brainstem evoked potentials (BAEPs), was performed on 34 patients with hereditary ataxias (HAs): 15 with Friedreich's disease (FD), 10 with early onset cerebellar ataxia (EOCA), and 9 with autosomal dominant cerebellar ataxia (ADCA). A higher incidence of abnormal central motor conduction was observed in FD than in EOCA patients, but was never observed in ADCA. A relationship between central motor conduction abnormalities and disease duration and clinical impairment was found only in FD patients. All FD patients showed severe impairment of the SSEPs that was not related to disease duration. In EOCA patients, less frequent and more variable SSEP abnormalities were observed. The lowest incidence of central SSEP abnormalities was observed in ADCA. The BAEP findings in all 3 groups of patients (but particularly those with EOCA) suggest prevalent brainstem damage.

Original languageEnglish
Pages (from-to)25-37
Number of pages13
JournalItalian Journal of Neurological Sciences
Issue number1
Publication statusPublished - Feb 1994


  • evoked potentials
  • inherited ataxias
  • motor cortex stimulation

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology


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