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Startle response: epileptic or non-epileptic? The case for "flash" SMA reflex seizures.
Epileptic Disord. 2001 Jan-Mar; 3(1):7-12.ED

Abstract

A 19-year-old woman complained of long-standing, frequent, debilitating 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 remission had 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.

Authors+Show Affiliations

Centre Saint-Paul, 13258 Marseille 09, France.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

11313216

Citation

Cokar, O, et al. "Startle Response: Epileptic or Non-epileptic? the Case for "flash" SMA Reflex Seizures." Epileptic Disorders : International Epilepsy Journal With Videotape, vol. 3, no. 1, 2001, pp. 7-12.
Cokar O, Gelisse P, Livet MO, et al. Startle response: epileptic or non-epileptic? The case for "flash" SMA reflex seizures. Epileptic Disord. 2001;3(1):7-12.
Cokar, O., Gelisse, P., Livet, M. O., Bureau, M., Habib, M., & Genton, P. (2001). Startle response: epileptic or non-epileptic? The case for "flash" SMA reflex seizures. Epileptic Disorders : International Epilepsy Journal With Videotape, 3(1), 7-12.
Cokar O, et al. Startle Response: Epileptic or Non-epileptic? the Case for "flash" SMA Reflex Seizures. Epileptic Disord. 2001 Jan-Mar;3(1):7-12. PubMed PMID: 11313216.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Startle response: epileptic or non-epileptic? The case for "flash" SMA reflex seizures. AU - Cokar,O, AU - Gelisse,P, AU - Livet,M O, AU - Bureau,M, AU - Habib,M, AU - Genton,P, PY - 2001/4/21/pubmed PY - 2001/8/24/medline PY - 2001/4/21/entrez SP - 7 EP - 12 JF - Epileptic disorders : international epilepsy journal with videotape JO - Epileptic Disord VL - 3 IS - 1 N2 - A 19-year-old woman complained of long-standing, frequent, debilitating 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 remission had 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. SN - 1294-9361 UR - https://www.unboundmedicine.com/medline/citation/11313216/Startle_response:_epileptic_or_non_epileptic_The_case_for_"flash"_SMA_reflex_seizures_ L2 - http://www.jle.com/medline.md?issn=1294-9361&vol=3&iss=1&page=7 DB - PRIME DP - Unbound Medicine ER -