Dr. Andrea Domenico Praticò, Department of Pediatrics, University of Catania, Via S. Sofia 78, Catania, Italy, phone: 00390953781193, fax: 0039095222532, e-mail:
Neurol Neurochir Pol. 2013 Nov-Dec;47(6):584-9. doi: 10.5114/ninp.2013.39076.
The term 'hemiconvulsion-hemiplegia-epilepsy syndrome' (HHE) was first used by Gastaut et al. to describe the se-quential combination of unilateral or predominantly unilateral clonic seizures (hemiconvulsion), occurring during the first 2 years of life, immediately followed by an ipsilateral flaccid hemiplegia lasting 7 or more days. In the following phase partial epileptic seizures occur. We report a case of HHE syndrome in a 3-year-old boy with partial seizures (hemiconvulsion lasting 15-30 minutes) followed by left hemiplegia and hyporeflexia. Magnetic resonance imaging showed diffuse and high signal hyperintensity of the whole right cerebral hemisphere. Diffusion-weighted images showed a reduction of the apparent diffusion coefficient in the subcortical region. Magnetic resonance arterio-graphy showed a narrow flow signal in the distal territory of the right middle cerebral artery. The authors emphasize the importance of neuroradiological findings in early diagnosis and in the follow-up of HHE syndrome.
“偏瘫-癫痫-偏瘫综合征”（HHE）一词最早由 Gastaut 等人用于描述单侧或主要单侧阵挛性发作（偏瘫）的序贯组合，发生在生命的前 2 年，紧接着同侧弛缓性偏瘫持续 7 天以上。在随后的阶段会出现部分癫痫发作。我们报告了一例 3 岁男孩的 HHE 综合征，表现为部分发作（偏瘫持续 15-30 分钟），随后出现左侧偏瘫和低反射。磁共振成像显示整个右大脑半球弥漫性和高信号高亮。弥散加权图像显示皮质下区域表观扩散系数降低。磁共振血管造影显示右侧大脑中动脉远端区域血流信号变窄。作者强调了神经影像学发现对 HHE 综合征早期诊断和随访的重要性。
