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老年人的伦诺克斯-加斯托综合征?

Lennox-Gastaut syndrome in the elderly?

作者信息

Benbadis S R, Dinner D S

机构信息

Department of Neurology, Cleveland Clinic Foundation, Ohio.

出版信息

Clin Electroencephalogr. 1994 Oct;25(4):142-7. doi: 10.1177/155005949402500406.

DOI:10.1177/155005949402500406
PMID:7813093
Abstract

The Lennox-Gastaut syndrome (LGS) is an electro-clinical syndrome of early childhood that consists of mental retardation, intractable generalized epilepsy with multiple seizure types, and typical EEG findings. We report a 64-year-old woman who was referred for presurgical evaluation with a diagnosis of intractable temporal lobe epilepsy since the age of 14 years, consisting of staring and unresponsiveness, sudden falls, and generalized convulsions. The average seizure frequency was 1-4 per month. Mental status and neurological examination were normal. There was no known etiology and magnetic resonance imaging (MRI) of the brain was normal. Interictal EEG showed findings characteristic of LGS: generalized slow spike-wave complexes, multifocal sharp waves, generalized polyspikes and paroxysmal fast activity during sleep. Despite the absence of mental retardation and the presence of normal alpha rhythm, the patient fulfills most diagnostic criteria for LGS, with unusually late onset and extremely delayed diagnosis. This case illustrates the controversial nosologic boundaries of the syndrome. Prolonged EEG video monitoring may be of value even in the elderly. The LGS and other varieties of secondary generalized epilepsy should be considered in the syndromic diagnosis of epilepsy in the elderly.

摘要

伦诺克斯 - 加斯托综合征(LGS)是一种儿童早期的电临床综合征,其特征包括智力发育迟缓、伴有多种发作类型的顽固性全身性癫痫以及典型的脑电图表现。我们报告了一名64岁女性,自14岁起就因顽固性颞叶癫痫被转诊进行术前评估,其发作表现为凝视和无反应、突然跌倒以及全身性惊厥。平均每月发作频率为1 - 4次。精神状态和神经系统检查均正常。病因不明,脑部磁共振成像(MRI)也正常。发作间期脑电图显示出LGS的特征性表现:全身性慢棘波综合波、多灶性尖波、全身性多棘波以及睡眠期间的阵发性快活动。尽管该患者没有智力发育迟缓且存在正常的α波节律,但她符合LGS的大多数诊断标准,只是发病时间异常晚且诊断极度延迟。这个病例说明了该综合征在疾病分类学上存在争议的界限。即使对于老年人,长时间的脑电图视频监测可能也有价值。在老年人癫痫的综合征诊断中,应考虑LGS和其他类型的继发性全身性癫痫。

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引用本文的文献

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Case Report: Late-Onset Lennox-Gastaut Syndrome Treated With Stereotactic Electroencephalography-Guided Radiofrequency Thermocoagulation Before Craniotomy.病例报告:开颅术前立体定向脑电图引导下射频热凝治疗迟发性Lennox-Gastaut综合征
Front Neurol. 2022 Jun 20;13:857767. doi: 10.3389/fneur.2022.857767. eCollection 2022.
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Brainstem dysfunction in patients with late-onset Lennox-Gastaut syndrome: Voxel-based morphometry and tract-based spatial statistics study.迟发性Lennox-Gastaut综合征患者的脑干功能障碍:基于体素的形态学测量和基于纤维束的空间统计学研究
Ann Indian Acad Neurol. 2016 Oct-Dec;19(4):518-522. doi: 10.4103/0972-2327.194462.