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Congenital Bilateral Perisylvian syndrome
Background
The term Congenital Bilateral Perisylvian syndrome (CBPS) describes a structural malformation of the brain. The underlying anomaly is Polymicrogyria, a malformation of the cerebral cortex (outer layer of the brain). The term polymicrogyria designates an excessive number of small and prominent convolutions spaced out by shallow and enlarged sulci (grooves), giving the surface of the brain a lumpy aspect. Although it may be difficult to recognise mild forms of polymicrogyria on a magnetic resonance imaging (MRI) scan, infolding of the outer layer of the brain and secondary, irregular, thickening due to packing of microgyri (small folds) represent quite distinctive MRI characteristics.
Polymicrogyria may have a focal or regional distribution or involve the whole cortical mantle (covering of the brain). There are consequently a wide spectrum of clinical manifestations which include children with severe encephalopathies (brain impairments) and intractable epilepsy, or normal individuals with selective impairment of cognitive functions (mental processes) in whom the mild cortical abnormality is only detected on pathological brain study.
Several malformation syndromes featuring bilateral polymicrogyria
have been described, including bilateral perisylvian polymicrogyria
(the most frequent form), bilateral parasagittal parietooccipital
polymicrogyria, bilateral frontal polymicrogyria and unilateral
perisylvian or multilobar polymicrogyria. Several distinct entities
might exist with regional distribution in which contiguous, non
overlapping areas of the cerebral cortex are involved, possibly under
the influence of regionally expressed developmental genes.
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