Document Detail


MR imaging of rhombencephalosynapsis: report of three cases and review of the literature.
MedLine Citation:
PMID:  1950929     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe the clinical and MR findings in three cases of rhombencephalosynapsis, a rare congenital malformation of the posterior fossa consisting of vermian agenesis or severe hypogenesis, fusion of the cerebellar hemispheres, and apposition or fusion of the dentate nuclei. Associated anomalies include hydrocephalus, fusion of the inferior colliculi, deficiency or absence of the septum pellucidum, and hypoplasia of the anterior commissure. Fourteen previous cases of rhombencephalosynapsis have been reported including Obersteiner's first report in 1914. The clinical presentation is variable, ranging from early death to variable degrees of cerebellar dysfunction and developmental delay. Patients may reach young adulthood. We report three additional cases and provide radiographic (MR) images of this unusual anomaly detected during life. Diagnoses in three children with rhombencephalosynapsis were made on the basis of MR findings. To our knowledge, this is the first report of this disorder being diagnosed in living patients.
Authors:
C L Truwit; A J Barkovich; R Shanahan; T V Maroldo
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  AJNR. American journal of neuroradiology     Volume:  12     ISSN:  0195-6108     ISO Abbreviation:  AJNR Am J Neuroradiol     Publication Date:    1991 Sep-Oct
Date Detail:
Created Date:  1991-12-17     Completed Date:  1991-12-17     Revised Date:  2008-02-14    
Medline Journal Info:
Nlm Unique ID:  8003708     Medline TA:  AJNR Am J Neuroradiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  957-65     Citation Subset:  IM    
Affiliation:
Department of Radiology, University of California, San Francisco 94143-0628.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis
Brain / abnormalities*,  pathology
Cerebellum / abnormalities
Child
Child, Preschool
Female
Hippocampus / abnormalities
Humans
Hydrocephalus / complications
Magnetic Resonance Imaging*
Male

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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