Harris C M, Boyd S, Chong K, Harkness W, Neville B G
Great Ormond Street Hospital for Children, NHS Trust and Institute of Child Health, University College London, UK.
J Neurol Sci. 1997 Oct 3;151(1):111-4. doi: 10.1016/s0022-510x(97)00102-0.
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.
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。