Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

Yekeler E., Ozmen M., Genchellac H., Dursun M., Acunas G.

PEDIATRIC RADIOLOGY, vol.34, no.11, pp.908-911, 2004 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 34 Issue: 11
  • Publication Date: 2004
  • Doi Number: 10.1007/s00247-004-1221-9
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.908-911
  • Istanbul University Affiliated: No


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.