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Front. Neurol. | doi: 10.3389/fneur.2019.01233

Three cases of hemiconvulsion-hemiplegia-epilepsy syndrome with focal cortical dysplasia type IIId

Shinji Itamura1,  Tohru Okanishi1*,  Yoshifumi Arai1,  Mitsuyo Nishimura1, Shimpei Baba1, Naoki Ichikawa1, Yoshimichi Hirayama2, Naoko Ishihara3, Takuya Hiraide4, Hidetoshi Ishigaki4, Tokiko Fukuda4, Yoshiro Otsuki1,  Hideo Enoki1 and  Ayataka Fujimoto1
  • 1Seirei Hamamatsu General Hospital, Japan
  • 2Naha Municipal Hospital, Japan
  • 3Fujita Health University, Japan
  • 4Hamamatsu University School of Medicine, Japan

Hemiconvulsion-hemiplegia-epilepsy syndrome (HHES) is a subset of acute encephalopathy characterized by infantile-onset with acute hemiconvulsive febrile status and subsequent unilateral cerebral atrophy and hemiparesis. In the chronic phase, patients with HHES develop epilepsy, typically displayed as intractable focal seizures. The patients are often intractable with antiepileptic drugs and need surgical treatment. Although viral encephalitis and genetic abnormalities are presumed to be the underlying etiology, the pathogenesis remains mostly unknown. We describe three cases of successful functional hemispherotomy for intractable epilepsy in HHES. Patients developed acute asymmetrical convulsive status following viral infections during the ages of 17 to 30 months. Their seizures were intractable with antiepileptic drugs and required hemispherotomy. On the basis of the pathological findings, all cases were diagnosed as focal cortical dysplasia type IIId. The epileptogenic mild cortical malformations may be the cause of HHES.

Keywords: Hemiconvulsion-hemiplegia-epilepsy syndrome, Hemispherotomy, Cortical malformation, Intractable epilepsy, epilepsy surgery, Pathology, histopathology, focal cortical dysplasia

Received: 19 Aug 2019; Accepted: 05 Nov 2019.

Copyright: © 2019 Itamura, Okanishi, Arai, Nishimura, Baba, Ichikawa, Hirayama, Ishihara, Hiraide, Ishigaki, Fukuda, Otsuki, Enoki and Fujimoto. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Dr. Tohru Okanishi, Seirei Hamamatsu General Hospital, Hamamatsu City, Japan,