Neonatal pontomedullary disconnection with aplasia or destruction of the lower brain stem: a case of pontoneocerebellar hypoplasia?
We report a neonate who presented with marked hypotonia and absent suck reflex. MR demonstrated complete absence of the pons as well as absence of a basilar artery flow void. Our case exhibits features similar to those described in previous reports of pontoneocerebellar hypoplasia, but with a more severe degree of pontine involvement. The associated vascular findings suggest a vascular insult to the brain stem as the cause.
Mamourian
A C
AC
Department of Radiology, Milton S. Hershey Medical Center, Hershey, Pa.
Miller
G
G
D001488:Basilar Artery / Q000473:pathology / Q000503:physiopathology; D001933:Brain Stem / Q000002:abnormalities* / Q000098:blood supply / Q000473:pathology; D002531:Cerebellum / Q000002:abnormalities* / Q000473:pathology; D002560:Cerebrovascular Circulation / Q000502:physiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D009123:Muscle Hypotonia / Q000503:physiopathology; D011149:Pons / Q000002:abnormalities* / Q000473:pathology; D013391:Sucking Behavior / Q000502:physiology; D014711:Vertebral Artery / Q000473:pathology / Q000503:physiopathology