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Brainstem calcification in Möbius syndrome
Joseph M Dooley1, Wendy A Stewart, James D Hayden
1Department of Pediatrics, Division of Pediatric Neurology, Dalhousie University and IWK Health Centre, Halifax, Nova Scotia, Canada.
Insights
Möbius syndrome, a congenital condition causing facial palsy, may stem from prenatal brain ischemia. This study found brainstem calcification in affected children, supporting the prenatal ischemia hypothesis.
Area of Science:
- Neurology
- Pediatrics
- Medical Genetics
Background:
- Möbius syndrome presents as congenital facial diplegia, often with limb or orofacial malformations.
- The exact pathogenesis remains unclear, with prenatal ischemia being a leading hypothesis.
Purpose of the Study:
- To investigate the underlying pathology of Möbius syndrome.
- To evaluate the association between Möbius syndrome and prenatal brain ischemia.
Main Methods:
- Retrospective review of seven children diagnosed with Möbius syndrome (1992-2001).
- Clinical assessment of facial palsy and associated malformations.
- Analysis of computed tomographic (CT) scans for brainstem abnormalities, particularly calcification.
Main Results:
- All seven patients exhibited incomplete bilateral facial palsy.
- Six patients presented with associated limb and/or orofacial anomalies.
- Five of six CT scans revealed brainstem calcification, predominantly in the floor of the fourth ventricle, present from early infancy.
Conclusions:
- The presence and static nature of brainstem calcification support a prenatal onset.
- Findings suggest that prenatal brain ischemia is a likely cause of Möbius syndrome's pathology.
Abstract:
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.
