P300 and respiratory findings in myotonic muscular dystrophy

Massimiliano Oliveri, Brigida Fierro, Rosalia Lo Presti, Filippo Brighina, Vincenzo La Bua, Gregorio Caimi

Research output: Contribution to journalArticlepeer-review


Ten patients with myotonic muscular dystrophy (MD) were examined by auditory event-related potentials (P300 ERPs), spirometric and blood gas analyses: arterial oxygen tension (PaO2), arterial carbon dioxide tension (PaCO2) and arterial oxygen saturation (SaO2). The aim of the study was to analyse the frequency of ERP abnormalities in this disease and to determine whether the neurophysiological evidence of cognitive impairment might be related to the ventilatory function abnormalities frequently described in MD. The mean P300 latency was significantly altered in MD patients compared with controls; P300 latencies did not correlate with spirometric parameters, blood gas values or with age, age at onset, duration or clinical status of the disease. This study provides neurophysiological evidence of cognitive impairment in MD patients. The cognitive deficits are not related to alveolar hypoventilation and appear to be a non progressive feature of the disease.

Original languageEnglish
Pages (from-to)149-154
Number of pages6
JournalFunctional Neurology
Issue number3
Publication statusPublished - 1999


  • Cognitive functions
  • Myotonic dystrophy
  • P300
  • Spirometry

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)


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