S turge– W eber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy
Abstract S turge– W eber 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 re...
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Published in | Brain pathology (Zurich, Switzerland) Vol. 25; no. 3; pp. 248 - 255 |
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Main Authors | , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
01.05.2015
|
Online Access | Get full text |
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Summary: | Abstract
S
turge–
W
eber 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. |
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ISSN: | 1015-6305 1750-3639 |
DOI: | 10.1111/bpa.12172 |