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阅读性癫痫的变异型。17例阅读诱发癫痫患者的临床及视频脑电图研究。

The variants of reading epilepsy. A clinical and video-EEG study of 17 patients with reading-induced seizures.

作者信息

Koutroumanidis M, Koepp M J, Richardson M P, Camfield C, Agathonikou A, Ried S, Papadimitriou A, Plant G T, Duncan J S, Panayiotopoulos C P

机构信息

Department of Clinical Neurophysiology and Epilepsies, St Thomas' Hospital, London, UK.

出版信息

Brain. 1998 Aug;121 ( Pt 8):1409-27. doi: 10.1093/brain/121.8.1409.

Abstract

We present the clinical and electrographic data of 17 patients with reading-induced seizures documented with ictal video-EEG studies during provocation with language related tasks. The median age at onset was 15 years (range 11-22 years) and the male:female ratio was 2.4. Fourteen patients had no spontaneous seizures of any type while the remaining three had infrequent generalized tonic-clonic seizures during nocturnal sleep. Two distinct electroclinical ictal patterns were confirmed on video-EEG analysis. (i) Fifteen patients had reading-induced jerks which invariably involved the region of the jaw but also included the upper limbs in five of them. Ictal EEG discharges were noted in 12 patients; these were brief but varied in terms of morphology and spatial distribution, with a clear tendency for left-sided predominance. All but one of these patients had similar myoclonic seizures induced by linguistic activities other than reading, the phenomenon probably justifying the term 'language-induced epilepsy'. Some patients had evidence of transient cognitive impairment associated with the reading-induced jaw or limb jerks. Three patients had a sibling with reading epilepsy but there was no other family history of epileptic seizures. (ii) Two patients had reading-provoked paroxysmal alexia without motor symptoms, associated with prolonged focal ictal EEG abnormalities. Reading provoked a subclinical, continuous and reproducible EEG activation over the left posterior temporal area. We propose that ictogenesis in reading or language-induced epilepsy is based on the reflex activation of a hyperexcitable network that subserves the function of speech and extends over multiple cerebral areas on both hemispheres. The parts of this network responding to the stimulus may, secondarily, drive the relative motor areas producing the typical regional myoclonus. This network hyperexcitability can be genetically determined and its clinical expression is age-related.

摘要

我们展示了17例阅读诱发癫痫患者的临床和脑电图数据,这些患者在语言相关任务诱发过程中通过发作期视频脑电图研究得以记录。发病的中位年龄为15岁(范围11 - 22岁),男女比例为2.4。14例患者无任何类型的自发癫痫发作,其余3例在夜间睡眠时有不频繁的全身强直阵挛发作。视频脑电图分析确认了两种不同的发作期电临床模式。(i)15例患者有阅读诱发的抽搐,总是累及下颌区域,但其中5例还包括上肢。12例患者记录到发作期脑电图放电;这些放电短暂,但形态和空间分布各异,明显倾向于左侧优势。除1例患者外,所有这些患者在阅读以外的语言活动时也会诱发类似的肌阵挛发作,这种现象可能解释了“语言诱发癫痫”这一术语。一些患者有与阅读诱发的下颌或肢体抽搐相关的短暂认知障碍证据。3例患者有患阅读性癫痫的兄弟姐妹,但无其他癫痫发作的家族史。(ii)2例患者有阅读诱发的阵发性失读症且无运动症状,伴有长时间的局灶性发作期脑电图异常。阅读在左侧颞后区域诱发亚临床、持续且可重复的脑电图激活。我们提出,阅读或语言诱发癫痫的发作机制基于一个超兴奋性网络的反射激活,该网络负责言语功能并延伸至双侧大脑的多个区域。该网络中对刺激作出反应的部分可能继而驱动相对应的运动区域,产生典型的局部肌阵挛。这种网络超兴奋性可能由遗传决定,其临床表型与年龄相关。

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