Hyperekplexia: A syndrome of pathological startle responeses

E. Sáenz‐Lope, F. J. Herranz‐Tanarro, Joseph C. Masdeu, J. R.Chacón Peña

Research output: Contribution to journalArticlepeer-review

83 Scopus citations


We describe a family and three sporadic cases of startle disease, or hyperekplexia. Sudden unexpected noises caused the patients to fall rigidly, often injuring themselves but retaining consciousness. This unusual entity differs from startle epilepsy and cataplexy. Clonazepam proved ineffective in three patients. Valproic acid, 5-hydroxytryptophan, or piracetam markedly reduced the abnormal startle in three patients.

Original languageEnglish (US)
Pages (from-to)36-41
Number of pages6
JournalAnnals of Neurology
Issue number1
StatePublished - Jan 1 1984

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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