BACKGROUND Hemiconvulsion-hemiplegia-epilepsy (HHE) involves infantile-onset acute hemiconvulsive febrile status epilepticus with subsequent unilateral cerebral atrophy and hemiparesis. Chronic epilepsy later develops, typically involving refractory focal seizures; however, the underlying pathophysiology of this epilepsy is not well understood. PATIENT We present a boy who had a typical acute presentation of HHE at 23 months, but an unusual evolution to chronic epilepsy in which the initially unaffected hemisphere was significantly abnormal. His initial acute presentation was right-sided hemiconvulsive febrile status epilepticus, with subsequent left cerebral hemiatrophy and hemiparesis affecting the right face, arm and leg. Focal seizures began at 5 years and were refractory to medical treatment. At 9 years, video EEG monitoring showed a striking pattern of interictal slow spike-wave and paroxysmal fast activity, maximal over the right, initially unaffected, hemisphere. He had primarily focal tonic seizures involving left-sided stiffening, also appearing to originate from the right hemisphere. Following left functional hemispherotomy he became seizure-free and parents reported improved cognitive function, attention and quality of life. DISCUSSION This boy had classic features of Lennox-Gastaut syndrome, but expressed almost exclusively over the right hemisphere, which was initially unaffected in his acute presentation of HHE. His evolution to "hemi-Lennox-Gastaut-like phenotype" illustrates the importance of monitoring chronic epilepsy in patients with HHE; early surgical intervention might prevent pathologic recruitment of bilateral secondary networks leading to the refractory seizures and cognitive impairment associated with Lennox-Gastaut syndrome.

中文翻译：

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半惊厥-偏瘫-癫痫演变为对侧半-Lennox-Gastaut样表型

背景半惊厥-偏瘫-癫痫 (HHE) 涉及婴儿起病的急性半惊厥性发热性癫痫持续状态，随后出现单侧脑萎缩和偏瘫。后来发展为慢性癫痫，通常涉及难治性局灶性癫痫发作；然而，这种癫痫的潜在病理生理学尚不清楚。患者 我们介绍了一个男孩，他在 23 个月时出现了典型的 HHE 急性表现，但异常演变为慢性癫痫，其中最初未受影响的大脑半球明显异常。他最初的急性表现是右侧半痉挛性发热性癫痫持续状态，随后出现左侧大脑半球萎缩和偏瘫，影响右侧面部、手臂和腿部。局灶性癫痫发作在 5 岁时开始，药物治疗无效。9岁时，视频脑电图监测显示发作间期慢棘波和阵发性快速活动的显着模式，最大在右侧，最初未受影响，半球。他的主要局灶性强直性癫痫发作涉及左侧僵硬，也似乎起源于右半球。在左侧功能性半球切开术之后，他没有癫痫发作，父母报告说认知功能、注意力和生活质量都有所改善。讨论 这个男孩具有 Lennox-Gastaut 综合征的典型特征，但几乎完全在右半球表达，这在他急性 HHE 表现中最初未受影响。他向“半-Lennox-Gastaut 样表型”的演变说明了监测 HHE 患者慢性癫痫的重要性；