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唐氏综合征成人癫痫的临床和脑电图特征。

Clinical and EEG features of seizures in adults with down syndrome.

机构信息

Department of Neurology and Neurophysiology, Cork University Hospital, Wilton, Cork, Ireland.

出版信息

J Clin Neurophysiol. 2011 Oct;28(5):469-73. doi: 10.1097/WNP.0b013e318230da76.

DOI:10.1097/WNP.0b013e318230da76
PMID:21946360
Abstract

In this study, the authors characterized the clinical and EEG features of adult patients with Down syndrome who were referred, for more than a 10-year period, to the Epilepsy Clinic in the Cork University Hospital. A retrospective audit of the charts of 28 patients with Down syndrome who had an EEG performed in the Cork University Hospital between January 1, 2000, and September 30, 2009, including clinical follow-up, was carried out. Demographics, age at first seizure, seizure types, EEG findings, antiepileptic drugs, psychoactive medications, and seizure control were documented. Seizures most commonly began in the fourth decade of life. Generalized tonic-clonic seizures were the most common type of seizures (15 patients, 54%), followed by complex partial seizures (5 patients, 18%) and generalized myoclonus (4 patients, 15%). A number of patients had more than one type of seizure. Diffuse background slowing was the dominant EEG abnormality seen in 17 patients (60%). Epileptiform activity was present in 5 patients (18%): 2 had focal epileptiform discharges in the frontal regions, 1 in the central region, 1 in the central posterior region bilaterally, and 1 in the temporal regions. The six patients who had poor seizure control were those who had seizure onset from the fourth decade onward and clinically had complex partial seizures in combination with either generalized tonic-clonic or generalized myoclonus; four of them had epileptiform activity on EEG, with two having focal frontal epileptiform discharges: one in the central posterior areas bilaterally and one in the temporal regions. Five of the 28 patients had a normal EEG and 3 of these had a seizure disorder. There was no correlation found between use of psychoactive drugs and increased frequency/poor seizure control in patients in our study. This study documents the variability of clinical and electrophysiologic features in a well-characterized cohort of patients with Down syndrome with adult-onset epilepsy.

摘要

在这项研究中,作者描述了在科克大学医院癫痫诊所就诊的 28 名唐氏综合征成年患者的临床和脑电图特征。对 2000 年 1 月 1 日至 2009 年 9 月 30 日期间在科克大学医院进行脑电图检查的 28 名唐氏综合征患者的图表进行了回顾性审核,包括临床随访。记录了人口统计学资料、首次发作年龄、发作类型、脑电图结果、抗癫痫药物、精神活性药物和发作控制情况。发作最常见于 40 岁以后。全身性强直阵挛发作是最常见的发作类型(15 例,54%),其次是复杂部分性发作(5 例,18%)和全身性肌阵挛(4 例,15%)。一些患者有多种类型的发作。17 例(60%)患者的脑电图以弥漫性背景减慢为主。5 例(18%)患者有癫痫样活动:2 例患者额区有局灶性癫痫样放电,1 例患者中央区有局灶性癫痫样放电,1 例患者双侧中央后区有局灶性癫痫样放电,1 例患者颞区有局灶性癫痫样放电。6 例癫痫控制不佳的患者是那些在 40 岁以后发病且临床为复杂部分性发作并伴有全身性强直阵挛或全身性肌阵挛的患者;其中 4 例脑电图有癫痫样活动,2 例有局灶性额区癫痫样放电:1 例双侧中央后区,1 例颞区。28 例患者中 5 例脑电图正常,其中 3 例有癫痫发作。在我们的研究中,没有发现精神活性药物的使用与患者的发作频率增加或控制不佳之间存在相关性。这项研究记录了一组具有明确特征的唐氏综合征成年患者癫痫发作的临床和电生理特征的变异性。

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