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婴儿期癫痫性眼球震颤

Epileptic nystagmus in infancy.

作者信息

Harris C M, Boyd S, Chong K, Harkness W, Neville B G

机构信息

Great Ormond Street Hospital for Children, NHS Trust and Institute of Child Health, University College London, UK.

出版信息

J Neurol Sci. 1997 Oct 3;151(1):111-4. doi: 10.1016/s0022-510x(97)00102-0.

Abstract

Epileptic nystagmus (EN) is a rare form of nystagmus that occurs only during epileptic seizures. We report an infantile case in which EN was first noted at 10 days of age. Electronystagmography showed a right-beating nystagmus with predominantly linear slow phases that traversed the midline. Neuro-imaging revealed dysplasia of the left middle temporal gyrus extending posteriorly into the parieto-occipital cortex. The right hemisphere and subcortical structures appeared normal. Perfusion studies demonstrated interictal hypoperfusion with ictal hyperperfusion in the left temporal lobe. Electrocorticography demonstrated spiking over the left temporal-parieto-occipital region. Following extensive surgical resection of this area and weaning of anti-convulsants, the child has remained seizure-free without nystagmus. This case demonstrates the cortical origin of EN, and shows that infant cortex has functioning efferent connections to brainstem oculomotor centres from 10 days of age.

摘要

癫痫性眼球震颤(EN)是一种罕见的眼球震颤形式,仅在癫痫发作时出现。我们报告一例婴儿病例,该患儿在10日龄时首次被发现患有EN。眼震电图显示为向右摆动的眼球震颤,主要为线性慢相,越过中线。神经影像学检查显示左颞中回发育异常,向后延伸至顶枕叶皮质。右侧半球和皮质下结构看起来正常。灌注研究显示左侧颞叶在发作间期灌注减低,发作期灌注增加。皮质脑电图显示左侧颞顶枕区有棘波。在对该区域进行广泛手术切除并停用抗惊厥药物后,患儿一直无癫痫发作且无眼球震颤。该病例证明了EN的皮质起源,并表明婴儿皮质从10日龄起就与脑干动眼神经中枢有功能性传出连接。

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