大型下丘脑错构瘤队列中的癫痫发作。

Epileptic Spasms in a Large Hypothalamic Hamartoma Cohort.

机构信息

Department of Pediatrics, Section of Pediatric Neurology and Developmental Neuroscience, 12298Baylor College of Medicine, Houston, TX, USA.

Department of Neurology and Neurophysiology, 12298Baylor College of Medicine, Houston, TX, USA.

出版信息

J Child Neurol. 2021 Mar;36(4):304-309. doi: 10.1177/0883073820968652. Epub 2020 Nov 10.

DOI:10.1177/0883073820968652

PMID:33170063

Abstract

INTRODUCTION

Hypothalamic hamartoma is rarely associated with epileptic spasms. We describe epileptic spasms in a large cohort of hypothalamic hamartoma patients.

METHODS

We performed a retrospective chart review between March 2011 and March 2020 to identify patients with hypothalamic hamartoma and epilepsy.

RESULTS

We identified 114 patients with hypothalamic hamartoma and epilepsy, only 3 male patients (2.6%) also had epileptic spasms. The epileptic spasms developed between 6 and 18 months of age. Epileptic spasms resolved with oral prednisolone in 1 and with vigabatrin in the second patient. The third patient continued epileptic spasms despite multiple antiepileptic drugs and partial resection of hypothalamic hamartoma. All 3 patients underwent laser-ablation of hypothalamic hamartoma at the age of 14, 29, and 63 months. The seizure burden decreased by 100%, 84%, and 93% at follow-up (3-47 months).

CONCLUSIONS

Epileptic spasms are rare in hypothalamic hamartoma patients and early laser-ablation could potentially treat epileptic spasms and all other seizure types associated with hypothalamic hamartoma.

摘要

介绍

下丘脑错构瘤很少与癫痫性痉挛有关。我们描述了一大组下丘脑错构瘤患者的癫痫性痉挛。

方法

我们对 2011 年 3 月至 2020 年 3 月期间的病历进行了回顾性分析，以确定患有下丘脑错构瘤和癫痫的患者。

结果

我们确定了 114 例下丘脑错构瘤合并癫痫的患者，只有 3 名男性患者（2.6%）也有癫痫性痉挛。癫痫性痉挛在 6 至 18 个月龄之间发生。1 例患者口服泼尼松龙，1 例患者使用氨己烯酸后癫痫性痉挛得到缓解。第 3 例患者尽管使用了多种抗癫痫药物并进行了下丘脑错构瘤部分切除，但仍持续癫痫性痉挛。所有 3 例患者均在 14、29 和 63 个月龄时接受了下丘脑错构瘤激光消融治疗。在随访（3-47 个月）时，癫痫发作的负担降低了 100%、84%和 93%。