HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) Data Supplement Data Supplement Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Verzijl, H. T.F.M. Articles by Padberg, G. W. Search for Related Content PubMed PubMed Citation Articles by Verzijl, H. T.F.M. Articles by Padberg, G. W. Related Collections Cranial neuropathy Anterior nerve cell disease

The neuropathology of hereditary congenital facial palsy vs Möbius syndrome

From the Departments of Neurology (Drs. Verzijl, Lammens, ten Donkelaar, and Padberg, and B. van der Zwaag) and Pathology (Dr. Lammens), 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, PO Box 9101, 6500 HB Nijmegen, The Netherlands; e-mail: h.verzijl{at}neuro.umcn.nl

Objective: To characterize the neuropathology of hereditary congenital facial palsy.

Methods: The authors compared brainstem pathology of three members of one family with autosomal dominant congenital facial palsy to that in three age-matched controls. The neuropathologic findings of the familial patients were compared with those of patients with Möbius syndrome.

Results: The authors observed a marked decrease in the number of neurons in the facial motor nucleus with corresponding small facial nerve remnants. In the patients with congenital facial palsy the number of facial motoneurons ranged between 280 and 1,680 as compared to 5,030 and 8,700 for controls. No signs of neuronal degeneration or necrosis with neuronal loss, gliosis, or calcifications were present. There were no other abnormalities of the rhombencephalon and its associated structures. The corticospinal tracts were fully developed. In contrast, Möbius syndrome is part of a more complex congenital anomaly of the posterior fossa with hypoplasia of the entire brainstem, including the traversing long tracts, with signs of neuronal degeneration and other congenital brain abnormalities.

Conclusion: Neuropathologic findings confirm clinical observations that hereditary congenital facial palsy and Möbius syndrome are two different entities with a different pathogenesis.

This article has been cited by other articles:

				L. Cattaneo, E. Chierici, B. Bianchi, E. Sesenna, and G. Pavesi The localization of facial motor impairment in sporadic Mobius syndrome Neurology, June 27, 2006; 66(12): 1907 - 1912. [Abstract] [Full Text] [PDF]

				The Neuropathology of Disorders of the Facial Nucleus Journal Watch Neurology, October 20, 2005; 2005(1020): 5 - 5. [Full Text]