| Dandy-Walker malformation masking the molar tooth sign: an illustrative case with magnetic resonance imaging follow-up. | |
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MedLine Citation:
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PMID: 20823032 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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Joubert syndrome is a disorder characterized by ataxia, developmental delay, oculomotor anomalies, and breathing irregularities, with cerebellar vermian and midbrain dysgenesis. The molar tooth sign, reflecting the midbrain dysgenesis of Joubert syndrome, is the neuroradiological hallmark and is an essential sign in the identification of this condition. Variable vermian agenesis, an expanded fourth ventricle, and a large posterior cranial fossa with a normal brainstem are typical of Dandy-Walker malformation. The authors report a case in which a Dandy-Walker malformation coexisted with Joubert syndrome, but initially prevented the ''molar tooth sign'' from being recognized because of an important cystic dilatation of the fourth ventricle. In this article, they discuss the importance of the re-examination of brain magnetic resonance features after decompression of the posterior cranial fossa in a patient with Dandy-Walker malformation and additional clinical neurological or systemic abnormalities typical of Joubert syndrome, to not miss the correct diagnosis. |
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Authors:
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Stefano Sartori; Kathrin Ludwig; Manuela Fortuna; Cinzia Marzocchi; Milena Calderone; Irene Toldo; Leonardo Salviati; Anna Maria Laverda; Romano Tenconi |
Publication Detail:
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Type: Journal Article Date: 2010-09-07 |
Journal Detail:
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Title: Journal of child neurology Volume: 25 ISSN: 1708-8283 ISO Abbreviation: J. Child Neurol. Publication Date: 2010 Nov |
Date Detail:
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Created Date: 2010-11-15 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8606714 Medline TA: J Child Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 1419-22 Citation Subset: IM |
Affiliation:
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Paediatric Neurology Unit, Department of Paediatrics Salus Pueri, University of Padua, Padua, Italy. ste.sartori@tin.it |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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