Document Detail


Abnormal myelination in Angelman syndrome.
MedLine Citation:
PMID:  18573670     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Patients with Angelman syndrome (OMIM # 105830) are generally thought to have normal brain imaging studies except for occasional minor cerebral atrophy. We report 9 patients with genetically proven Angelman syndrome, who were examined by magnetic resonance imaging (MRI) between the ages of 7.5 months and 5 years. MRI in the 5 patients examined during infancy revealed myelination delay and a deficit of white matter. Retarded and/or abnormal myelination in Angelman syndrome seems to be a common finding that may be diagnostically misleading. This is particularly important in the evaluation of infants with possible Angelman syndrome, who present with nonspecific clinical features and have not yet developed the characteristic behavioural, language, and movement abnormalities.
Authors:
Inga Harting; Angelika Seitz; Dietz Rating; Klaus Sartor; Johannes Zschocke; Bart Janssen; Friedrich Ebinger; Nicole I Wolf
Publication Detail:
Type:  Journal Article     Date:  2008-06-24
Journal Detail:
Title:  European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society     Volume:  13     ISSN:  1532-2130     ISO Abbreviation:  Eur. J. Paediatr. Neurol.     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-04-17     Completed Date:  2009-07-01     Revised Date:  2010-04-16    
Medline Journal Info:
Nlm Unique ID:  9715169     Medline TA:  Eur J Paediatr Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  271-6     Citation Subset:  IM    
Affiliation:
Department of Neuroradiology, University Hospital Heidelberg, Germany. inga.harting@med.uni-heidelberg.de
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MeSH Terms
Descriptor/Qualifier:
Angelman Syndrome / diagnosis,  pathology*
Brain / pathology*
Child, Preschool
Developmental Disabilities / diagnosis,  pathology*
Female
Humans
Image Processing, Computer-Assisted
Infant
Magnetic Resonance Imaging
Male
Nerve Fibers, Myelinated / pathology*
Retrospective Studies
Comments/Corrections
Comment In:
Eur J Paediatr Neurol. 2010 May;14(3):292   [PMID:  19720548 ]

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