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单侧多脑叶和半球多小脑回畸形(PMG)相关癫痫的临床特征及不同治疗方案的发作转归。

Clinical features of unilateral multilobar and hemispheric polymicrogyria (PMG)-related epilepsy and seizure outcome with different treatment options.

机构信息

Department of Pediatrics, Peking University First Hospital, Beijing, China.

Pediatric Epilepsy Center, Peking University First Hospital, Beijing, China.

出版信息

Epilepsia Open. 2024 Aug;9(4):1480-1492. doi: 10.1002/epi4.12988. Epub 2024 Jun 19.

DOI:10.1002/epi4.12988
PMID:38898786
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11296091/
Abstract

OBJECTIVE

To provide evidence for choosing surgical or nonsurgical treatment for epilepsy in patients with unilateral multilobar and hemispheric polymicrogyria (PMG).

METHODS

We searched published studies until September 2022 related to unilateral multilobar and hemispheric PMG and included patients who were followed up at the Pediatric Epilepsy Centre of Peking University First Hospital in the past 10 years. We summarized the clinical characteristics and compared the long-term outcomes after surgical or nonsurgical (anti-seizure medications, ASMs) treatment.

RESULTS

A total of 70 patients (49 surgical, 21 non-surgical) with unilateral multilobar and hemispheric PMG were included. The median age at epilepsy onset was 2.5 years (1.0-4.1). The most common seizure types were focal and atypical absence seizures. In the whole cohort, 87.3% had hemiparesis and 67.1% had electrical status epilepticus during slow sleep (ESES). There were significant differences in age at epilepsy onset, extent of lesion, and EEG interictal discharges between the two groups. At the last follow-up (median 14.1 years), the rates of seizure-freedom (81.6% vs. 57.1%, p = 0.032) and ASM discontinuation (44.4% vs. 6.3%, p = 0.006) were higher in the surgical group than in the nonsurgical group. Patients in the surgical group had a higher rate of seizure-freedom with complete resection/disconnection than with subtotal resection (87.5% vs. 55.6%, p = 0.078), but with no statistically significant difference. In the nonsurgical group, more extensive lesions were associated with worse seizure outcomes. Cognition improved postoperatively in 90% of surgical patients.

SIGNIFICANCE

In patients with unilateral multilobar and hemispheric PMG, the age of seizure onset, the extent of the lesion and EEG features can help determine whether surgery should be performed early. Additionally, surgery could be more favorable for achieving seizure freedom and cognitive improvement sooner.

PLAIN LANGUAGE SUMMARY

We aim to summarize clinical characteristics and compare the long-term outcomes after surgical and nonsurgical (ASM) treatment to provide a basis for treatment decisions for patients with unilateral multilobar and hemispheric polymicrogyria (PMG)-related epilepsy. We found that patients with unilateral hemispheric and multilobar PMG had significantly higher rates of seizure freedom and ASM discontinuation with surgical treatment than with nonsurgical treatment. In the surgical group, seizure outcomes were better in patients treated with complete resection/disconnection than in those treated with subtotal resection, but the difference was not statistically significant.

摘要

目的

为单侧多脑叶和半球性多微脑回(PMG)患者选择手术或非手术治疗癫痫提供证据。

方法

我们检索了截至 2022 年 9 月发表的与单侧多脑叶和半球性 PMG 相关的研究,并纳入了过去 10 年在北京大学第一医院儿科癫痫中心随访的患者。我们总结了临床特征,并比较了手术和非手术(抗癫痫药物,ASMs)治疗后的长期结果。

结果

共纳入 70 例单侧多脑叶和半球性 PMG 患者(49 例手术,21 例非手术)。癫痫发作的中位年龄为 2.5 岁(1.0-4.1)。最常见的发作类型为局灶性和非典型失神发作。在整个队列中,87.3%有偏瘫,67.1%有睡眠中电持续状态(ESES)。两组之间在癫痫发作年龄、病变范围和脑电图间发性放电方面存在显著差异。末次随访(中位时间 14.1 年),手术组的无癫痫发作率(81.6% vs. 57.1%,p=0.032)和停用 ASM 率(44.4% vs. 6.3%,p=0.006)均高于非手术组。手术组完全切除/离断的无癫痫发作率(87.5% vs. 55.6%,p=0.078)高于次全切除,但差异无统计学意义。在非手术组中,更广泛的病变与更差的癫痫发作结局相关。90%的手术患者术后认知功能改善。

意义

对于单侧多脑叶和半球性 PMG 患者,癫痫发作年龄、病变范围和脑电图特征有助于确定是否应早期进行手术。此外,手术可能更有利于更早地实现无癫痫发作和认知改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6598/11296091/eedc3af22c9a/EPI4-9-1480-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6598/11296091/8c2a61ebb848/EPI4-9-1480-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6598/11296091/eedc3af22c9a/EPI4-9-1480-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6598/11296091/8c2a61ebb848/EPI4-9-1480-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6598/11296091/eedc3af22c9a/EPI4-9-1480-g002.jpg

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