Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId.
cortical malformation
epilepsy surgery
focal cortical dysplasia
hemiconvulsion-hemiplegia-epilepsy syndrome
hemispherotomy
histopathology
intractable epilepsy
pathology
Journal
Frontiers in neurology
ISSN: 1664-2295
Titre abrégé: Front Neurol
Pays: Switzerland
ID NLM: 101546899
Informations de publication
Date de publication:
2019
2019
Historique:
received:
19
08
2019
accepted:
05
11
2019
entrez:
12
12
2019
pubmed:
12
12
2019
medline:
12
12
2019
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 31824410
doi: 10.3389/fneur.2019.01233
pmc: PMC6879674
doi:
Types de publication
Case Reports
Langues
eng
Pagination
1233Informations de copyright
Copyright © 2019 Itamura, Okanishi, Arai, Nishimura, Baba, Ichikawa, Hirayama, Ishihara, Hiraide, Ishigaki, Fukuda, Otsuki, Enoki and Fujimoto.
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