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Titolo:
Startle response: epileptic or non-epileptic? The case for "flash" SMA reflex seizures
Autore:
Cokar, O; Gelisse, P; Livet, MO; Bureau, M; Habib, M; Genton, P;
Indirizzi:
Ctr St Paul, F-13258 Marseille 09, France Ctr St Paul Marseille France 09 tr St Paul, F-13258 Marseille 09, France Hop Nord, Marseille, France Hop Nord Marseille FranceHop Nord, Marseille, France
Titolo Testata:
EPILEPTIC DISORDERS
fascicolo: 1, volume: 3, anno: 2001,
pagine: 7 - 11
SICI:
1294-9361(200101/03)3:1<7:SREONT>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
INHIBITORY GLYCINE RECEPTOR; HYPEREKPLEXIA; MUTATIONS; DISEASE; SUBUNIT;
Keywords:
startle epilepsy; reflex epilepsy; hyperekplexia; supplementary motor area;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
18
Recensione:
Indirizzi per estratti:
Indirizzo: Genton, P Ctr St Paul, F-13258 Marseille 09, France Ctr St Paul Marseille France 09 , F-13258 Marseille 09, France
Citazione:
O. Cokar et al., "Startle response: epileptic or non-epileptic? The case for "flash" SMA reflex seizures", EPILEPT DIS, 3(1), 2001, pp. 7-11

Abstract

A 19-year-old woman complained of long-standing, frequent, debilatating brusque movements triggered by unexpected stimuli. She was neurologically normal and neuroimaging was also normal. Conspicuous startle reactions were easily reproduced under EEG and video monitoring: the interictal EEG was normal, the ictal recordings doubtful; clinically, the startle reaction was asymmetric, with elevation of the left limbs. The diagnosis of hyperekplexia and startle epilepsy were discussed. We learned that she had been evaluated at age 3-4 months for spontaneous, generalised tonic-clonic seizures and "infantile spasms", in fact for early-onset startle reactions triggered by noise or contact, in association with prominent EEG changes. A full remissionhad been achieved under ACTH therapy, but the startle reactions had reappeared at around age six. The patient was successfully treated with carbamazepine. The history, clinical and neurophysiological data led us to discuss the diagnosis of hyperekplexia and startle epilepsy. We concluded that the patient had an unusual form of cryptogenic focal epilepsy originating from the supplementary motor area, presenting as strictly stimulus-triggered "flash" seizures.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/03/20 alle ore 15:37:21