Familial Startle Disease (Hyperexplexia): Electrophysiologic Studies

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39 Scopus citations


Six affected members from a family of 15 patients with familial startle disease (hyperexplexia) underwent extensive electrophysiologic evaluation. The most marked abnormality consisted of prominent C response 60 to 75 ms after median and peroneal nerve stimulation. The somatosensory evoked responses were also relatively high in amplitude. These findings suggest that hyperactive long-loop reflexes may constitute the physiologic basis of startle disease.

Original languageEnglish (US)
Pages (from-to)71-74
Number of pages4
JournalArchives of Neurology
Issue number1
StatePublished - Jan 1984

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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