| The neuropathology of hereditary congenital facial palsy vs Möbius syndrome. | |
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MedLine Citation:
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PMID: 15728286 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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H T F M Verzijl; B van der Zwaag; M Lammens; H J ten Donkelaar; G W Padberg |
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Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Neurology Volume: 64 ISSN: 1526-632X ISO Abbreviation: Neurology Publication Date: 2005 Feb |
Date Detail:
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Created Date: 2005-02-24 Completed Date: 2005-10-18 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0401060 Medline TA: Neurology Country: United States |
Other Details:
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Languages: eng Pagination: 649-53 Citation Subset: AIM; IM |
Affiliation:
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Department of Neurology, University Medical Center Nijmegen, PO Box 9101, 6500 HB Nijmegen, The Netherlands. h.verzijl@neuro.umcn.nl |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple
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pathology Adult Aged Aged, 80 and over Brain / pathology Brain Stem / pathology* Case-Control Studies Cell Count Facial Nerve / pathology* Facial Paralysis / complications, genetics, pathology* Female Genes, Dominant Humans Infant, Newborn Male Meningitis, Bacterial / complications, pathology Mobius Syndrome / genetics, pathology* Motor Neurons / pathology* Nerve Degeneration Pyramidal Tracts / pathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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