title: Startle disease or hyperekplexia: adolescent onset and response to valproate.
abstract: We report a boy with rigidity and hyperekplexia or startle disease which began in adolescence. His symptoms completely resolved with valproic acid therapy. Two relatives have the minor form of this disorder, suggesting autosomal dominant inheritance with variable penetrance.
authors.affiliation: Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada.
authors.forename: J M
authors.initials: JM
authors.lastname: Dooley
authors.forename: F
authors.initials: F
authors.lastname: Andermann
mesh_terms: D000293:Adolescent; D004827:Epilepsy / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D008297:Male; D013216:Reflex, Startle / Q000187:drug effects* / Q000502:physiology; D014635:Valproic Acid / Q000627:therapeutic use*
chemical_list: D014635:Valproic Acid