Myers Kenneth A, Scheffer Ingrid E, Archer John S
Epilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia; Department of Pediatrics, Faculty of Medicine, McGill University, Montreal, Quebec, Canada; Division of Child Neurology, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada.
Epilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia; The Florey Institute of Neuroscience and Mental Health, Heidelberg, Victoria, Australia; Department of Paediatrics, The University of Melbourne, Parkville, Victoria, Australia; Department of Neurology, Royal Children's Hospital, Parkville, Victoria, Australia.
Brain Dev. 2018 May;40(5):425-428. doi: 10.1016/j.braindev.2018.01.005. Epub 2018 Feb 16.
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.
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.
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.
偏瘫-偏瘫-癫痫（HHE）包括婴儿期起病的急性偏瘫性热性惊厥持续状态，随后出现单侧脑萎缩和偏瘫。慢性癫痫随后发展，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未完全了解。
我们报告一名23个月大的男孩，他有典型的HHE急性表现，但向慢性癫痫的演变不寻常，其中最初未受影响的半球明显异常。他最初的急性表现是右侧偏瘫性热性惊厥持续状态，随后出现左侧脑半球萎缩和影响右侧面部、手臂和腿部的偏瘫。局灶性癫痫发作始于5岁，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活动的显著模式，在右侧最初未受影响的半球最为明显。他主要有涉及左侧僵硬的局灶性强直发作，似乎也起源于右侧半球。在进行左侧功能性大脑半球切除术之后，他不再发作，父母报告其认知功能、注意力和生活质量有所改善。
这名男孩具有Lennox-Gastaut综合征的典型特征，但几乎完全表现在右侧半球，该半球在他HHE的急性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；早期手术干预可能会防止双侧继发性网络的病理性募集，从而导致与Lennox-Gastaut综合征相关的难治性癫痫发作和认知障碍。
