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儿童伴情感性发作的自限性癫痫:一种明确的癫痫综合征?

Self-limited epilepsy of childhood with affective seizures: A well-defined epileptic syndrome?

机构信息

Hospital Público Materno Infantil de Salta, Argentina.

Hospital de Niños Victor J Vilela de Rosario, Santa Fé, Argentina.

出版信息

Epilepsy Behav. 2021 Apr;117:107885. doi: 10.1016/j.yebeh.2021.107885. Epub 2021 Mar 11.

DOI:10.1016/j.yebeh.2021.107885
PMID:33714932
Abstract

OBJECTIVE

Here we present cases of focal epilepsy with affective symptoms analyzing seizure characteristics, EEG pattern, treatment, and outcome.

METHODS

A multicenter, descriptive, retrospective study was conducted evaluating 18 patients with self-limited epilepsy who presented with seizures with affective symptoms seen between April 2000 and April 2018 at eight Argentinian centers.

RESULTS

Eighteen patients had focal seizures with affective symptoms; all of them had affective symptoms characterized by sudden fright or terror and screaming. Seizures started with manifestations of sudden fright or terror manifested by a facial expression of fear; consciousness was mildly impaired in 15/18 patients. Eleven of the patients also had autonomic manifestations, such as pallor, sweating, and abdominal pain. In addition, four of these 11 patients had ictus emeticus and one also presented with unilateral deviation of the eyes and head. Speech arrest, salivation, glottal noises, and chewing or swallowing movements were observed in 2/18 patients at the onset of the affective seizures. Two others also had mild asymmetric dystonic seizures involving both hands and arms. Three patients had tonic deviation of the mouth involving the lips and tongue as well pharyngeal and laryngeal muscles, resulting in anarthria and drooling. Two patients had brief hemifacial focal clonic seizures.

CONCLUSION

Affective manifestations associated or not with motor and/or autonomic manifestations and associated with typical EEG features of the idiopathic focal epilepsies of childhood is a particular presentation of self-limited focal epilepsy in childhood.

摘要

目的

本研究分析了伴有情感症状的局灶性癫痫病例,总结了发作特征、脑电图模式、治疗和预后。

方法

采用多中心、描述性、回顾性研究方法,评估了 2000 年 4 月至 2018 年 4 月期间在 8 个阿根廷中心就诊的 18 例具有自限性癫痫且伴有情感症状的局灶性癫痫患者。

结果

18 例患者均为局灶性癫痫伴情感症状,表现为突然惊恐或尖叫;15/18 例患者意识轻度受损,所有患者均有情感症状,表现为惊恐面容;11 例患者还伴有自主神经表现,如面色苍白、出汗和腹痛;此外,这 11 例患者中有 4 例出现癫痫性呕吐,1 例出现单侧眼球和头部偏斜。在 18 例患者中,2 例在情感性发作开始时出现言语停止、流涎、喉音和咀嚼或吞咽运动,2 例还有轻微的双侧手和手臂的扭转痉挛发作;3 例患者出现口角歪斜、构音障碍和流涎,伴有口唇和舌部、咽及喉部肌肉的强直,3 例患者伴有短暂的面肌局灶性阵挛发作。

结论

伴有或不伴有运动和/或自主神经症状且伴有儿童特发性局灶性癫痫典型脑电图特征的情感表现是儿童自限性局灶性癫痫的一种特殊表现。

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