Brainstem calcification in Möbius syndrome.
Möbius syndrome is characterized by congenital facial diplegia, and may be associated with limb or orofacial malformations. A number of mechanisms have been proposed to explain the pathogenesis, including prenatal ischemia. We identified seven children with Möbius syndrome over the 10-year interval 1992-2001, all of whom manifested incomplete bilateral facial palsy. Associated limb and orofacial anomalies were observed in six cases. Computed tomographic scans were available in six children, and five of them manifested brainstem calcification which was most prominent in the floor of the fourth ventricle. The calcification was detected as early as 7 days of age and did not change with time, suggesting a static condition of prenatal onset. These observations support the hypothesis that the pathology in Möbius syndrome is secondary to prenatal brain ischemia.
Dooley
Joseph M
JM
Department of Pediatrics, Division of Pediatric Neurology, Dalhousie University and IWK Health Centre, Halifax, Nova Scotia, Canada.
Stewart
Wendy A
WA
Hayden
James D
JD
Therrien
Arawn
A
D001933:Brain Stem / Q000000981:diagnostic imaging / Q000473:pathology*; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000473:pathology*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D020331:Mobius Syndrome / Q000000981:diagnostic imaging / Q000473:pathology*; D014057:Tomography, X-Ray Computed / Q000379:methods