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Möbius syndrome redefined

A syndrome of rhombencephalic maldevelopment

From the Departments of Neurology (Drs. Verzijl, van der Zwaag, and Padberg) and Ophthalmology (Dr. Cruysberg), University Medical Center Nijmegen, The Netherlands.

Address correspondence and reprint requests to Dr. H.T.F.M. Verzijl, Department of Neurology, University Medical Center Nijmegen, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands; e-mail: H.Verzijl{at}neuro.umcn.nl

Objective: To investigate the variable clinical picture of Möbius syndrome (MIM no. 157900) and to further understand the pathogenesis of the disorder.

Methods: A standardized questionnaire was submitted to 37 Dutch patients with Möbius syndrome. All underwent standardized neurologic examination with special attention to cranial nerve functions, motor skills, and facial and limb anomalies.

Results: Of 37 patients with facial paresis, 97% had bilateral and 3% had unilateral ocular abduction weakness. Further analysis showed isolated abducens nerve palsy in 9%, a conjugated horizontal gaze paresis in 48%, features of Duane retraction syndrome in 34%, and congenital fibrosis of the extraocular muscles in 9%. Other signs included lingual involvement (77%), dysfunction of palate and pharynx (56%), general motor disability (88%), poor coordination (83%), and respiratory abnormalities (19%).

Conclusion: Möbius syndrome is more than a cranial nerve or nuclear developmental disorder. It is a syndrome of rhombencephalic maldevelopment involving predominantly motor nuclei and axons, as well as traversing long tracts. The authors also noted gaze palsies, Duane retraction syndrome, feeding and respiratory problems, and poor motor development, suggesting a regional developmental disorder.

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