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Hyperekplexia: A syndrome of pathological startle responeses
Author(s) -
SáenzLope E.,
HerranzTanarro F. J.,
Masdeu J. C.,
Peña J. R. Chacón
Publication year - 1984
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410150107
Subject(s) - clonazepam , moro reflex , pathological , psychology , valproic acid , medicine , epilepsy , anesthesia , neuroscience , reflex
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.