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结节性硬化症患儿亚临床癫痫发作的演变

The Evolution of Subclinical Seizures in Children With Tuberous Sclerosis Complex.

作者信息

de Groen Anne-Elise C, Bolton Jeffrey, Bergin Ann Marie, Sahin Mustafa, Peters Jurriaan M

机构信息

1 Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.

2 Department of Neurology, Translational Neuroscience Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.

出版信息

J Child Neurol. 2019 Oct;34(12):770-777. doi: 10.1177/0883073819860640. Epub 2019 Jul 10.

Abstract

BACKGROUND

Subclinical seizures are electrographic seizures that present without subjective or objective clinical symptoms. In tuberous sclerosis complex, it is not known whether subclinical seizures occur alone, forewarn, or coexist with clinical seizures. To address this knowledge gap, we studied the prevalence and evolution of subclinical seizures in tuberous sclerosis complex.

METHODS

We retrospectively reviewed electroencephalography (EEG) data from our tuberous sclerosis complex clinic with subclinical seizures and clinical seizures in a blinded fashion. Based on EEG location and ictal pattern, subclinical seizures were classified as having a clinical counterpart from the same epileptogenic region (match) or not (no match).

RESULTS

Of 208 children with tuberous sclerosis complex, 138 had epilepsy and available EEG data. Subclinical seizures were detected in 26 of 138 (19%) children. Twenty-four children had both subclinical seizures and clinical seizures captured on EEG. In 13 of 24, subclinical seizures were detected as a novel, not previously recorded seizure type. In these children, subclinical seizures preceded matching clinical seizures in 4 (31%) within a median time of 4.5 months (range 2-14), whereas 9 (69%) never had any matching clinical seizure. In 11 of 24 children, subclinical seizures were not novel and could be matched to a previously recorded clinical seizure. Matching seizure types were focal (n = 10, 67%), tonic (n = 2), epileptic spasms (n = 2), and status epilepticus (n = 1).

CONCLUSIONS

Subclinical seizures occur in one-fifth of children with tuberous sclerosis complex and epilepsy, and match with clinical seizures in a small majority. In a third of patients presenting with a novel subclinical seizure, matching clinical seizures follow.

摘要

背景

亚临床发作是指无主观或客观临床症状的脑电图发作。在结节性硬化症中,尚不清楚亚临床发作是单独出现、作为先兆还是与临床发作共存。为填补这一知识空白,我们研究了结节性硬化症中亚临床发作的患病率和演变情况。

方法

我们以盲法回顾性分析了来自我们结节性硬化症诊所的脑电图(EEG)数据,这些数据涉及亚临床发作和临床发作。根据EEG定位和发作模式,将亚临床发作分为与同一致痫区域的临床发作相对应(匹配)或不相对应(不匹配)。

结果

在208例结节性硬化症患儿中,138例患有癫痫且有可用EEG数据。138例患儿中有26例(19%)检测到亚临床发作。24例患儿的EEG记录到了亚临床发作和临床发作。在24例中的13例中,亚临床发作被检测为一种新的、以前未记录过的发作类型。在这些患儿中,4例(31%)的亚临床发作在中位时间4.5个月(范围2 - 14个月)内先于匹配的临床发作出现,而9例(69%)从未有过任何匹配的临床发作。在24例患儿中的11例中,亚临床发作并非新出现的,可与先前记录的临床发作相匹配。匹配的发作类型为局灶性(n = 10,6

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