Hyperekplexia: A syndrome of pathological startle responeses

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

doi:10.1002/ana.410150107

Hyperekplexia: A syndrome of pathological startle responeses

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

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Abstract

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 language	English (US)
Pages (from-to)	36-41
Number of pages	6
Journal	Annals of Neurology
Volume	15
Issue number	1
DOIs	https://doi.org/10.1002/ana.410150107
State	Published - Jan 1984

ASJC Scopus subject areas

Neurology
Clinical Neurology

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AB - 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.

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Hyperekplexia: A syndrome of pathological startle responeses

Abstract

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