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伴失神发作的眼睑肌阵挛(杰文斯综合征):一种明确的特发性全身性癫痫综合征还是一系列光敏性疾病?

Eyelid myoclonia with absences (Jeavons syndrome): a well-defined idiopathic generalized epilepsy syndrome or a spectrum of photosensitive conditions?

作者信息

Striano Salvatore, Capovilla Giuseppe, Sofia Vito, Romeo Antonino, Rubboli Guido, Striano Pasquale, Trenité Dorothée Kasteleijn-Nolst

机构信息

Epilepsy Center, Department of Neurological Sciences, Federico II University, Napoli, Italy.

出版信息

Epilepsia. 2009 May;50 Suppl 5:15-9. doi: 10.1111/j.1528-1167.2009.02114.x.

Abstract

Eyelid myoclonia with absences (EMA), or Jeavons syndrome, is a generalized epileptic condition clinically characterized by eyelid myoclonia (EM) with or without absences, eye closure-induced electroencephalography (EEG) paroxysms, and photosensitivity; in addition, rare tonic-clonic seizures may also occur. Although first described in 1977 and widely reported by several authors within the last few years, EMA has not been yet recognized as a definite epileptic syndrome. However, when strict criteria are applied to the diagnosis, EMA appears to be a distinctive condition that could be considered a myoclonic epileptic syndrome, with myoclonia limited to the eyelids, rather than an epileptic syndrome with absences.

摘要

眼睑肌阵挛伴失神发作(EMA),即杰冯斯综合征,是一种全身性癫痫疾病,临床特征为伴有或不伴有失神发作的眼睑肌阵挛(EM)、闭眼诱发的脑电图(EEG)阵发性活动以及光敏性;此外,还可能罕见地出现强直阵挛发作。尽管EMA于1977年首次被描述,且在过去几年中有多位作者广泛报道,但它尚未被确认为一种明确的癫痫综合征。然而,当应用严格的诊断标准时,EMA似乎是一种独特的疾病,可被视为一种肌阵挛性癫痫综合征,其肌阵挛仅限于眼睑,而非伴有失神发作的癫痫综合征。

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