Department of Neurology, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.
Department of Neurology, Clinica San Lucas, Neuquen, Argentina.
Epilepsy Behav. 2022 Jan;126:108451. doi: 10.1016/j.yebeh.2021.108451. Epub 2021 Dec 1.
We studied cases with long-lasting epileptic spasms (ES) considered as a spasm status analyzing type of epilepsy, epileptic syndrome, etiology, treatment, and outcome in 21 patients.
We evaluated the charts of 21 patients seen between June 2006 and July 2017 who met the electroclinical diagnostic criteria of a spasm status. The spasm status was defined as continuous ES lasting 30 min or longer.
The type of ES was mixed in nine patients, flexion in seven, and extension in five. Epileptic spasms were asymmetric in three patients and unilateral in two. They occurred on awakening in all patients, while during sleep they decreased in all and disappeared in three cases. The duration of the spasm status ranged from 40 min to 15 days according to the seizure diaries of patients and video-EEG recordings. Two well-defined subgroups of patients were recognized; the first included patients with West syndrome (WS) and the second other types of severe non-West epilepsy syndromes. The spasm status responded well to oral vigabatrin (VGB) in four patients, oral topiramate (TPM) in three, oral corticosteroids in one, and cannabidiol in another patient. A good response was observed with benzodiazepines in six patients, with phenytoin (PH) in two, and with phenobarbital (PB) in one. Adrenocorticotropic hormone (ACTH) was effective in one patient and the ketogenic diet in two. Prognosis depends on the etiology.
In this study we identified patients with WS and other types of severe non-West epilepsy syndromes who had a particular type of long-lasting ES that, in spite of its long duration does not strictly meet the criteria of the International League against Epilepsy (ILAE) classification of status epilepticus, may be considered a spasm status.
我们研究了被认为是癫痫持续状态的持续性癫痫痉挛（ES）病例，分析了其癫痫类型、癫痫综合征、病因、治疗和结局。
我们评估了 21 名患者的病历，这些患者于 2006 年 6 月至 2017 年 7 月间就诊，符合癫痫持续状态的临床电诊断标准。癫痫持续状态定义为持续 30 分钟或更长时间的痉挛性癫痫发作。
9 名患者的痉挛类型为混合性，7 名患者为屈肌性，5 名患者为伸肌性。3 名患者的痉挛不对称，2 名患者为单侧。所有患者的痉挛发作均发生在觉醒时，而在睡眠时，所有患者的痉挛发作减少，3 名患者的痉挛发作消失。根据患者的发作日记和视频脑电图记录，痉挛持续状态的持续时间从 40 分钟到 15 天不等。我们识别出了两组具有明确特征的患者；第一组包括 West 综合征（WS）患者，第二组包括其他类型的严重非 West 癫痫综合征患者。4 名患者口服氨己烯酸（VGB）、3 名患者口服托吡酯（TPM）、1 名患者口服皮质类固醇、1 名患者口服大麻二醇反应良好。6 名患者对苯二氮䓬类药物反应良好，2 名患者对苯妥英（PH）反应良好，1 名患者对苯巴比妥（PB）反应良好。促肾上腺皮质激素（ACTH）对 1 名患者有效，生酮饮食对 2 名患者有效。预后取决于病因。
在这项研究中，我们发现了患有 WS 和其他类型的严重非 West 癫痫综合征的患者，他们有一种特殊类型的持续性癫痫痉挛，尽管持续时间较长，但并不严格符合国际抗癫痫联盟（ILAE）癫痫持续状态分类标准，可被视为痉挛状态。
