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

Shinji Itamura, Tohru Okanishi, Yoshifumi Arai, Mitsuyo Nishimura, Shimpei Baba, Naoki Ichikawa, Yoshimichi Hirayama, Naoko Ishihara, Takuya Hiraide, Hidetoshi Ishigaki, Tokiko Fukuda, Yoshiro Otsuki, Hideo Enoki, Ayataka Fujimoto

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4 Citations (Scopus)


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.

Original languageEnglish
Article number1233
JournalFrontiers in Neurology
Publication statusPublished - 20-11-2019

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology


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