Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia:: A report with pathophysiological implications
Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia:: A report with pathophysiological implications
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DOI：
10.1016/j.seizure.2007.01.009
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发表时间：
2007-06-01
影响因子：
3
通讯作者：
Vallee, Louis
中科院分区：
文献类型：
--
作者：
Auvin, Stephane;Devisme, Louise;Vallee, Louis
偏侧惊厥-偏瘫-癫痫综合征（HHE）的发病机制尚不清楚。目前提出的致病机制是静脉血栓形成和/或缺氧诱导的神经元损伤。以前的异常的大脑被建议为潜在mechanism.Materials和方法：我们报告一个病人谁提出了急性偏侧痉挛偏瘫（HH）综合征，但不幸死亡。我们讨论了可能的病理生理学HH综合征和可能的治疗意义，利用神经影像学和病理学研究的数据。尸检进行了包括免疫组织化学和电子显微镜的脑组织。结果：在扩散加权成像的异常表明，癫痫半球的细胞毒性水肿。病理学检查证实右侧半球水肿，无任何畸形、炎症、感染或代谢性疾病。我们发现在右侧丘脑轴突损伤证实了抗神经丝染色。讨论：病理研究表明，细胞毒性水肿是负责神经元损伤。在HH综合征中，有两种机制在晚期癫痫的发展中起作用，这可能表明细胞毒性水肿和/或丘脑功能障碍引起的丘脑-皮质回路中断诱导的延迟性细胞死亡。在急性表现中，应讨论使用抗水肿治疗以防止细胞损伤。(c)2007年英国癫痫协会。由爱思唯尔有限公司出版。保留所有权利。
The mechanisms underlying the hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) remain unclear. The current proposed pathogenic mechanism is a neuronal injury induced by venous thrombosis and/or hypoxia. Previous abnormalities of the brain were suggested as underlying mechanism.Materials and methods: We report a patient who presented acutely with hemiconvulsion-hemiplegia (HH) syndrome, but unfortunately died. We discuss the possible pathophysiology of the HH syndrome and possible therapeutic implications utilizing the data from neuroimaging and pathological studies. Post-mortem examination was performed including immunohistochemistry and electron microscopy of the brain tissue.Results: The abnormalities in diffusion-weighted imaging indicate cytotoxic edema of, the epileptic hemisphere. The pathological studies confirmed a right homogenous hemispheric edema without evidence of any malformation, inflammatory, infectious or metabolic disease. We found axonal damages in the right thalamus confirmed by anti-neurofilament staining.Discussion: The, pathological studies suggest that cytotoxic edema is responsible for neuronal damage. In HH syndrome, two mechanisms playing a role in the development of a later epilepsy could suggest delayed cell death induced by cytotoxic edema and/or thalamic dysfunction causing a disruption of thalamo-cortical circuit. In acute presentation, the use of anti-edema therapy should be discussed to prevent the cell injury. (c) 2007 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.