Oe H, Yuasa R, Tsuchiyama M, Kitamura K
Department of Neurology, Sakai Municipal Hospital.
Rinsho Shinkeigaku. 1999 Apr;39(4):485-8.
A 32-year-old man with hemiconvulsions, hemiplegia, epilepsy (HHE) syndrome is described. He was well developed with a normal pregnancy and delivery, but at age 10 months, he had status epilepticus during a febrile illness. Thereafter, he was noted to have left hemiparesis and mental retardation with recurrent hemiconvulsions. Magnetic resonance (MR) images showed atrophy and degeneration of the right cerebral cortex and white matter, homolateral thalamus, caudate nucleus, and hippocampus, with hyperintensities in both T2-weighted (TR/2200, TE/90) and proton (TR/2200, TE/30) images. There were also slight bilateral cerebellar atrophies. Quantitative single photon emission computed tomographic (SPECT) images using technetium-99m-ethyl cysteinate dimer (99mTc-ECD) revealed markedly reduced cerebral blood flow (CBF) in the right cerebral hemisphere, homolateral thalamus, caudate nucleus and bilateral cerebellum. Bilateral putamen and the medial occipital lobe showed normal findings on MR images and normal regional CBF in SPECT images. We suppose these selective neuronal injures in this case of HHE syndrome will be mainly due to histotoxic factors in epileptic brain damage.
本文描述了一名患有偏瘫性惊厥、偏瘫、癫痫（HHE）综合征的32岁男性。他发育良好，孕期及分娩正常，但在10个月大时，因发热性疾病出现癫痫持续状态。此后，他被发现有左侧偏瘫和智力发育迟缓，并伴有反复的偏瘫性惊厥。磁共振（MR）图像显示右侧大脑皮质、白质、同侧丘脑、尾状核和海马萎缩及变性，在T2加权（TR/2200，TE/90）和质子（TR/2200，TE/30）图像上均有高信号。双侧小脑也有轻度萎缩。使用锝-99m-乙基半胱氨酸二聚体（99mTc-ECD）的定量单光子发射计算机断层扫描（SPECT）图像显示，右侧大脑半球、同侧丘脑、尾状核和双侧小脑的脑血流量（CBF）明显减少。双侧壳核和枕叶内侧在MR图像上表现正常，在SPECT图像上区域脑血流量也正常。我们推测，该例HHE综合征中的这些选择性神经元损伤主要归因于癫痫脑损伤中的组织毒性因素。
