Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis.
Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented.
Yekeler
Ensar
E
Department of Radiology, Istanbul Faculty of Medicine, Istanbul 90, Turkey. ensaryekeler@hotmail.com
Ozmen
Meral
M
Genchellac
Hakan
H
Dursun
Memduh
M
Acunas
Gulden
G
D000293:Adolescent; D002540:Cerebral Cortex / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000473:pathology; D004827:Epilepsy; D005260:Female; D006801:Humans; D008607:Intellectual Disability; D008279:Magnetic Resonance Imaging; D010291:Paresis; D010902:Pituitary Gland / Q000002:abnormalities*; D013577:Syndrome; D014057:Tomography, X-Ray Computed