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成人Lennox-Gastaut综合征:38例患者的长期临床随访及其发作记录分析

Lennox-Gastaut syndrome in adulthood: Long-term clinical follow-up of 38 patients and analysis of their recorded seizures.

作者信息

Vignoli Aglaia, Oggioni Gaia, De Maria Giovanni, Peron Angela, Savini Miriam Nella, Zambrelli Elena, Chiesa Valentina, La Briola Francesca, Turner Katherine, Canevini Maria Paola

机构信息

Epilepsy Center, San Paolo Hospital, Milan, Italy; Department of Health Sciences, Università degli Studi di Milano, Italy.

Epilepsy Center, San Paolo Hospital, Milan, Italy.

出版信息

Epilepsy Behav. 2017 Dec;77:73-78. doi: 10.1016/j.yebeh.2017.09.006. Epub 2017 Nov 7.

Abstract

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy with childhood onset that usually continues through adolescence and into adulthood. In the long term, patients with this condition still have intractable seizures, intellectual disability, behavioral problems, and physical comorbidities. The aim of this study was to describe the clinical and EEG characteristics of a group of adults with Lennox-Gastaut syndrome. We identified 38 (22 females, 16 males) patients with LGS older than age 18years at their last evaluation, with mean age of 43.3±10.6years. Median follow-up was 14.4years (range: 2-40). All of our patients had 3 or more seizure types during their clinical history. The most prevalent seizure types at follow-up were atypical absences (28/38), tonic (28/38), generalized tonic-clonic (17/38), focal (11/38), and myoclonic seizures (9/38). All patients had drug-resistant seizures. Besides epilepsy, intellectual disability and behavioral problems were prominent features. Surprisingly, paroxysmal nonepileptic seizures were reported in 3 patients. Our observations confirm the poor outcome of Lennox-Gastaut syndrome through adulthood, regardless of age at seizure onset, etiology, and history of previous West syndrome.

摘要

Lennox-Gastaut综合征(LGS)是一种儿童期起病的严重癫痫性脑病,通常会持续到青少年期及成年期。从长远来看,患有这种疾病的患者仍有难治性癫痫发作、智力残疾、行为问题和躯体合并症。本研究的目的是描述一组成年Lennox-Gastaut综合征患者的临床和脑电图特征。我们确定了38例(22例女性,16例男性)在最后一次评估时年龄超过18岁的LGS患者,平均年龄为43.3±10.6岁。中位随访时间为14.4年(范围:2-40年)。我们所有的患者在临床病史中都有3种或更多的癫痫发作类型。随访期间最常见的癫痫发作类型是非典型失神发作(28/38)、强直发作(28/38)、全身强直-阵挛发作(17/38)、局灶性发作(11/38)和肌阵挛发作(9/38)。所有患者都有药物难治性癫痫发作。除癫痫外,智力残疾和行为问题也是突出特征。令人惊讶的是,3例患者报告有阵发性非癫痫性发作。我们的观察结果证实,无论癫痫发作起病年龄、病因以及既往West综合征病史如何,Lennox-Gastaut综合征成年后的预后都很差。

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