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Focal cortical dysplasia type IIa underlying epileptogenesis in patients with epilepsy associated with Sturge‐Weber syndrome
Author(s) -
Murakami Nobuya,
Morioka Takato,
Suzuki Satoshi O.,
Hashiguchi Kimiaki,
Amano Toshiyuki,
Sakata Ayumi,
Iwaki Toru,
Sasaki Tomio
Publication year - 2012
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1167.2012.03628.x
Subject(s) - epileptogenesis , cortical dysplasia , angiomatosis , sturge–weber syndrome , epilepsy , ictal , medicine , pathology , angioma , epilepsy surgery , neuroscience , psychology , vascular disease , surgery , psychiatry
Summary In patients with epilepsy associated with Sturge‐Weber syndrome (SWS), epileptogenesis has been suggested to be caused by chronic ischemia in cortical areas affected by leptomeningeal angiomatosis or by ischemia‐related cortical malformations. However, this has not been fully verified electrophysiologically. We herein present two cases of SWS with medically intractable epilepsy in which the epileptogenic area involved focal cortical dysplasia (FCD) type IIa near the region of leptomeningeal angiomatosis. In both cases, the ictal‐onset zones were identified by chronic subdural electrodes, and the presence of FCD type IIa was shown histopathologically. In SWS, especially in association with focal leptomeningeal angiomatosis, FCD may thus play a major role in epileptogenesis. FCD should therefore be demonstrated by the collective findings of perioperative neurophysiologic examination, anatomic and functional neuroimaging, and histopathologic examination.