Brain, Vol 121, Issue 12 2229-2238, Copyright © 1998 by Oxford University Press
P Van Bogaert, P David, CA Gillain, D Wikler, P Damhaut, E Scalais, C Nuttin, C Wetzburger, HB Szliwowski, T Metens and S Goldman
We studied 10 patients who had neurological disorders with a MRI-based
diagnosis of perisylvian dysgenesis based on the fact that the parasagittal
and centrifugal extremity of the sylvian fissure was abnormally mesial.
This abnormality was bilateral in seven cases; in the other three patients,
the contralateral sylvian fissure appeared either normal (two cases) or
enlarged (open operculum). The perisylvian cortex had a polymicrogyric
appearance in most patients. Potential aetiopathogenic factors were
determined in four patients. In two of them, ischaemia at mid-gestation was
ascribed to death of a co-twin in a context of monozygotic twinning. In the
other two patients, who were siblings, genetic factors were suspected.
Pseudobulbar palsy was found in eight patients and epilepsy in five
patients. We used PET with [18F]fluorodeoxyglucose to test the hypothesis
that, despite this clinical and MRI heterogeneity, regional cerebral
glucose distribution could have common features in these patients. The
analysis of PET data was performed by visual inspection in two cases and by
using statistical parametric mapping (SPM) in eight patients compared with
a control group. Segmented grey matter MRIs of seven out these patients
were also analysed using SPM. We found that the abnormal perisylvian cortex
had normal grey matter activity in eight patients and in the other two
there was a heterogeneous pattern with areas of preserved metabolism and of
decreased metabolism. Metabolic changes were also detected outside the
polymicrogyric-like cortex; three patients had hypometabolic areas in
cortical regions where the MRI appeared normal and had a normal intensity.
When polymicrogyria extended into the white matter, this ectopic dysgenetic
cortex was associated with a grey matter pattern within the white matter
territory, and was detected by SPM as areas of PET hypermetabolism and MRI
hyperintensity. In order to detect possible metabolic changes undetected by
the individual analyses, the group of patients was compared with the
control group. This comparison revealed bilateral hypometabolism in the
frontal opercular cortex. We propose that these PET data be considered in
light of the presumed cyto-architectonic pattern of perisylvian dysgenesis,
i.e. polymicrogyria. In this malformation, two dense cell layers are
separated by a necrotic sparse cell layer. We speculate that the amount of
synaptic activity preserved in these dense cell layers depends on the
importance and timing of the necrotic process; this hypothesis accounts for
the large range of metabolic patterns found, from profoundly decreased
glucose metabolism to nearly normal activity.
ARTICLES
Perisylvian dysgenesis. Clinical, EEG, MRI and glucose metabolism features in 10 patients
Department of Pediatric Neurology, Hopital Erasme, Universite Libre de Bruxelles, Brussels, Belgium.
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