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痴笑性癫痫发作的发作期症状学

Ictal semiology of gelastic seizures.

作者信息

Mirandola Laura, Cantalupo Gaetano, d'Orsi Giuseppe, Meletti Stefano, Vaudano Anna Elisabetta, Di Vito Lidia, Vignoli Aglaia, Tassi Laura, Pelliccia Veronica

机构信息

Neurology Unit, San Giovanni Bosco Hospital, Turin, Italy.

Child Neuropsychiatry Unit, Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, University of Verona, Verona, Italy; Research Center for Pediatric Epilepsies Verona, Italy.

出版信息

Epilepsy Behav. 2023 Mar;140:109025. doi: 10.1016/j.yebeh.2022.109025. Epub 2023 Feb 11.

Abstract

Gelastic seizures are rare epileptic manifestations characterized by laughter or a smile. The main etiology is represented by hypothalamic hamartoma, but also focal localization of the epileptogenic zone is described. We reviewed a group of patients with gelastic seizures to describe the semiology and to establish any difference related to diverse epilepsy etiologies. Thirty-five seizures from 16 patients (6 females) were reviewed. The study confirms that hypothalamic hamartoma is the more frequent etiology associated with gelastic seizures. Laughter represented the majority of gelastic ictal signs, while the ictal smile was less frequent. In 87.5% of patients, the manifestation of laughter or smile was the only ictal phenomenon, or the first and the most important clinical sign. Interestingly, it has been observed that patients with a lesion localized in the hypothalamic region had more frequently laughter with emotional involvement and that laughter was the only manifestation of the seizure. On the contrary, patients with lesions localized outside the hypothalamic region had more often seizures with laugh without emotional involvement, resembling a more mechanical action, and associated with other semeiological signs. It, therefore, seems possible to assume that the emotional involvement and the expression of mirth during the seizure, especially in children, are more frequently associated with hypothalamic hamartoma. On the contrary, when the semiology includes less conveyed emotion similar to a mechanical action and other symptoms, an extra hypothalamic localization should be considered.

摘要

痴笑性癫痫发作是一种罕见的癫痫表现形式,其特征为大笑或微笑。主要病因是下丘脑错构瘤,但也有癫痫发作起源灶局灶性定位的描述。我们回顾了一组痴笑性癫痫发作患者,以描述其症状学,并确定与不同癫痫病因相关的差异。对16例患者(6名女性)的35次发作进行了回顾。该研究证实,下丘脑错构瘤是与痴笑性癫痫发作相关的最常见病因。大笑是痴笑性发作期体征的主要表现,而发作期微笑则较少见。在87.5%的患者中,大笑或微笑的表现是唯一的发作期现象,或是首要且最重要的临床体征。有趣的是,观察发现下丘脑区域有病变的患者更常出现伴有情感参与的大笑,且大笑是发作的唯一表现。相反,下丘脑区域以外有病变的患者更常出现无情感参与的大笑发作,类似一种更机械的动作,并伴有其他症状学体征。因此,似乎可以推测,发作期间的情感参与和欢笑表达,尤其是在儿童中,更常与下丘脑错构瘤相关。相反,当症状学包括较少传达情感、类似机械动作以及其他症状时,应考虑下丘脑外定位。

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