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115 例患儿半脑切除术后的长期功能预后及其预测因素。

Long-term functional outcomes and their predictors after hemispherectomy in 115 children.

机构信息

Department of Neurology, Section of Pediatric Epilepsy, Epilepsy Center, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, U.S.A.

出版信息

Epilepsia. 2013 Oct;54(10):1771-9. doi: 10.1111/epi.12342. Epub 2013 Aug 23.

Abstract

PURPOSE

To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had hemispherectomy. Functional outcome measures studied were the following: ambulation ability, visual symptoms, spoken language, reading skills, and behavioral problems.

METHODS

We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center. Preoperative clinical, electroencephalography (EEG), imaging, and surgical data were collected. One hundred twenty-five families completed a structured questionnaire to assess the functional status and seizure outcome. Prognostic predictors were examined using a multivariate regression analysis.

KEY FINDINGS

At a mean follow-up of 6.05 years after hemispherectomy, 70 patients (56%) were seizure-free and 45 (36%) had seizure recurrence; 10 patients (8%) were free of their preoperative seizures but had new-onset nonepileptic spells and were excluded from further analysis. Of 115, at follow-up (mean age at follow-up 12.7 years, range 2-28 years), 96 patients (83%) walked independently, 10 (8.7%) walked with assistance, and 9 (7.8%) were unable to walk. New visual symptoms that were not present preoperatively were reported only in 28 patients (24%). Eighty patients (70%) had satisfactory spoken language skills but only 44 (42%) of the 105 children older than 6 years had satisfactory reading skills. Significant behavioral problems were reported in 30 patients (27%). Only five (6.2%) of the 81 children aged between 6 and 18 years attended mainstream school without assistance; 48 (59%) were in mainstream school with assistance and the rest were in special school for disabled or home cared. Five (21%) of the 24 patients older than 18 years of age were gainfully employed. Multivariate logistic regression analysis identified the following factors as independently associated with poor functional outcome. (1) Seizure recurrence negatively affected all functional domains--ambulation ability, spoken language and reading skills, and behavior (p < 0.05). (2) Abnormalities in the unoperated hemisphere on magnetic resonance imaging (MRI) (p < 0.05) and preexisting quadriparesis (p < 0.01) correlated with poor motor outcome. (3) Multilobar MRI abnormalities in the contralateral hemisphere (odds ratio [OR] = 13.9, p = 0.001) and young age (indeterminate preoperative language status) at hemispherectomy (OR = 11.1, p = 0.01) also correlated with poor language outcome. (4) Younger age at epilepsy onset correlated with poor reading skills (p = 0.01) but not with spoken language skills.

SIGNIFICANCE

This study highlights the long-term functional status of patients after hemispherectomy. The majority of patients were ambulant independently; however, impairments in reading and spoken language were frequent. Seizure recurrence after hemispherectomy and contralateral hemisphere abnormalities on MRI were the major predictors of poor outcome in ambulation, spoken language, and reading abilities. This study will assist in presurgical counseling using simple understandable functional outcome measures and may help in planning early interventions after hemispherectomy to improve functional outcome.

摘要

目的

采用以患者/家庭为中心的方法,研究一组接受半球切除术的儿童的长期功能结果及其预测因素。研究的功能结果测量包括以下方面:步行能力、视觉症状、口语语言、阅读技能和行为问题。

方法

我们回顾了 1997 年至 2009 年期间在我们中心接受半球切除术的 186 例连续儿童。收集了术前临床、脑电图(EEG)、影像学和手术数据。125 个家庭完成了一份结构化问卷,以评估功能状态和癫痫发作结果。使用多元回归分析检查预后预测因素。

主要发现

在半球切除术 6.05 年后的平均随访中,70 例(56%)患者无癫痫发作,45 例(36%)癫痫发作复发;10 例(8%)患者无术前癫痫发作,但出现新的非癫痫发作,并被排除在进一步分析之外。在 115 例可随访的患者中(随访时平均年龄 12.7 岁,范围 2-28 岁),96 例(83%)能够独立行走,10 例(8.7%)需要辅助行走,9 例(7.8%)无法行走。仅 28 例患者(24%)报告出现术前不存在的新视觉症状。80 例(70%)患者口语语言技能良好,但只有 44 例(42%)年龄大于 6 岁的儿童具有满意的阅读技能。30 例患者(27%)存在明显的行为问题。在 81 名年龄在 6 至 18 岁之间的儿童中,只有 5 名(6.2%)在没有帮助的情况下就读主流学校;48 名(59%)在主流学校接受辅助教育,其余的在特殊学校或在家接受教育。24 名年龄大于 18 岁的患者中,有 5 名(21%)有收入。多元逻辑回归分析确定以下因素与不良功能结果独立相关。(1)癫痫发作复发对所有功能领域——步行能力、口语语言和阅读技能以及行为都有负面影响(p<0.05)。(2)磁共振成像(MRI)上未手术半球的异常(p<0.05)和术前四肢瘫痪(p<0.01)与运动功能不良相关。(3)对侧半球多脑叶 MRI 异常(优势比[OR] = 13.9,p = 0.001)和半球切除术时年龄较小(术前语言状态不确定)(OR = 11.1,p = 0.01)也与语言功能不良相关。(4)癫痫发作发病年龄较小与阅读技能较差相关(p = 0.01),但与口语语言技能无关。

意义

这项研究强调了患者半球切除术 6.05 年后的长期功能状况。大多数患者能够独立行走;然而,阅读和口语语言方面的障碍较为常见。半球切除术后继发性癫痫发作和对侧半球 MRI 异常是步行、口语语言和阅读能力不良预后的主要预测因素。这项研究将有助于使用简单易懂的功能结果测量进行术前咨询,并可能有助于计划半球切除术术后早期干预,以改善功能结果。

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