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Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId

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...

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Autores principales: Itamura, Shinji, Okanishi, Tohru, Arai, Yoshifumi, Nishimura, Mitsuyo, Baba, Shimpei, Ichikawa, Naoki, Hirayama, Yoshimichi, Ishihara, Naoko, Hiraide, Takuya, Ishigaki, Hidetoshi, Fukuda, Tokiko, Otsuki, Yoshiro, Enoki, Hideo, Fujimoto, Ayataka
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6879674/
https://www.ncbi.nlm.nih.gov/pubmed/31824410
http://dx.doi.org/10.3389/fneur.2019.01233
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author Itamura, Shinji
Okanishi, Tohru
Arai, Yoshifumi
Nishimura, Mitsuyo
Baba, Shimpei
Ichikawa, Naoki
Hirayama, Yoshimichi
Ishihara, Naoko
Hiraide, Takuya
Ishigaki, Hidetoshi
Fukuda, Tokiko
Otsuki, Yoshiro
Enoki, Hideo
Fujimoto, Ayataka
author_facet Itamura, Shinji
Okanishi, Tohru
Arai, Yoshifumi
Nishimura, Mitsuyo
Baba, Shimpei
Ichikawa, Naoki
Hirayama, Yoshimichi
Ishihara, Naoko
Hiraide, Takuya
Ishigaki, Hidetoshi
Fukuda, Tokiko
Otsuki, Yoshiro
Enoki, Hideo
Fujimoto, Ayataka
author_sort Itamura, Shinji
collection PubMed
description 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 17–30 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 (FCD) type IIId. The epileptogenic mild cortical malformations may be the cause of HHES.
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spelling pubmed-68796742019-12-10 Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId Itamura, Shinji Okanishi, Tohru Arai, Yoshifumi Nishimura, Mitsuyo Baba, Shimpei Ichikawa, Naoki Hirayama, Yoshimichi Ishihara, Naoko Hiraide, Takuya Ishigaki, Hidetoshi Fukuda, Tokiko Otsuki, Yoshiro Enoki, Hideo Fujimoto, Ayataka Front Neurol Neurology 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 17–30 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 (FCD) type IIId. The epileptogenic mild cortical malformations may be the cause of HHES. Frontiers Media S.A. 2019-11-20 /pmc/articles/PMC6879674/ /pubmed/31824410 http://dx.doi.org/10.3389/fneur.2019.01233 Text en Copyright © 2019 Itamura, Okanishi, Arai, Nishimura, Baba, Ichikawa, Hirayama, Ishihara, Hiraide, Ishigaki, Fukuda, Otsuki, Enoki and Fujimoto. http://creativecommons.org/licenses/by/4.0/ 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.
spellingShingle Neurology
Itamura, Shinji
Okanishi, Tohru
Arai, Yoshifumi
Nishimura, Mitsuyo
Baba, Shimpei
Ichikawa, Naoki
Hirayama, Yoshimichi
Ishihara, Naoko
Hiraide, Takuya
Ishigaki, Hidetoshi
Fukuda, Tokiko
Otsuki, Yoshiro
Enoki, Hideo
Fujimoto, Ayataka
Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
title Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
title_full Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
title_fullStr Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
title_full_unstemmed Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
title_short Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
title_sort three cases of hemiconvulsion-hemiplegia-epilepsy syndrome with focal cortical dysplasia type iiid
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6879674/
https://www.ncbi.nlm.nih.gov/pubmed/31824410
http://dx.doi.org/10.3389/fneur.2019.01233
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