Congenital bilateral perisylvian syndrome (CBPS): do concomitant esophageal malformations indicate a poor prognosis?
Congenital bilateral perisylvian syndrome (CBPS) is a syndrome of cortical malformation characterized by faciopharyngoglossomasticatory diplegia. We report on two cases of CBPS with associated esophageal malformations and a poor mental and motor development. The association of CBPS and esophageal malformations may indicate a subgroup of patients with a very early prenatal injury, characterised by a bad prognosis due to severe cortical disorganization. However, it can not be excluded that the association of CBPS and esophageal malformation is purely coincidental.
Küker
W
W
Department of Neuroradiology, University Hospital, Tübingen, Germany. wmkueker@med.uni-tuebingen.de
Friese
S
S
Riethmüller
J
J
Krägeloh-Mann
I
I
D000015:Abnormalities, Multiple / Q000473:pathology*; D002540:Cerebral Cortex / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology; D003680:Deglutition Disorders / Q000209:etiology / Q000473:pathology; D002658:Developmental Disabilities / Q000209:etiology; D004947:Esophagus / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019957:Motor Skills Disorders / Q000209:etiology; D011379:Prognosis; D013577:Syndrome