Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy

Wang DD, Blümcke I, Coras R, Zhou WJ, Lu DH, Gui QP, Hu JX, Zuo HC, Chen SY, Piao YS (2015)

Publication Type: Journal article

Publication year: 2015

Journal

Brain Pathology Wiley-Blackwell

Book Volume: 25

Pages Range: 248-55

Journal Issue: 3

DOI: 10.1111/bpa.12172

Abstract

Sturge-Weber syndrome (SWS) is a rare syndrome characterized by capillary-venous malformations involving skin and brain. Many patients with SWS also suffer from drug-resistant epilepsy. We retrospectively studied a series of six SWS patients with epilepsy and extensive neurosurgical resections. At time of surgery, the patients' age ranged from 11 to 35 years (with a mean of 20.2 years). All surgical specimens were well preserved, which allowed a systematic microscopical inspection utilizing the 2011 ILAE classification for focal cortical dysplasia (FCD). Neuropathology revealed dysmorphic-like neurons with hypertrophic cell bodies reminiscent to those described for FCD type IIa in all cases. However, gross architectural abnormalities of neocortical layering typical for FCD type IIa were missing, and we propose to classify this pattern as FCD ILAE type IIIc. In addition, our patients with earliest seizure onset also showed polymicrogyria (PMG; n = 4). The ictal onset zones were identified in all patients by subdural electrodes, and these areas always showed histopathological evidence for FCD type IIIc. Four out of five patients had favorable seizure control after surgery with a mean follow-up period of 1.7 years. We concluded from our study that FCD type IIIc and PMG are frequently associated findings in SWS. FCD type IIIc may play a major epileptogenic role in SWS and complete resection of the associated FCD should be considered a prognostic key factor to achieve seizure control.

Authors with CRIS profile

Ingmar Blümcke Lehrstuhl für Neuropathologie

Involved external institutions

Capital University of Medical Sciences / 首都医科大学 CN China (CN) Tsinghua University CN China (CN) People's Liberation Army Hospital / 中国人民解放军总医院 CN China (CN)

How to cite

APA:

Wang, D.-D., Blümcke, I., Coras, R., Zhou, W.-J., Lu, D.-H., Gui, Q.-P.,... Piao, Y.-S. (2015). Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy. Brain Pathology, 25(3), 248-55. https://doi.org/10.1111/bpa.12172

MLA:

Wang, Dan-Dan, et al. "Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy." Brain Pathology 25.3 (2015): 248-55.

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