Document Detail


Abnormalities of the brain in nonketotic hyperglycinemia: MR manifestations.
MedLine Citation:
PMID:  2494850     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
MR imaging in seven patients (4 days to 38 months old) with nonketotic hyperglycinemia showed age-related findings of progressive atrophy and delayed myelination. Parenchymal volume loss was found as early as 4 days after birth and increased in severity with increasing age to 27 months. Both supratentorial and infratentorial volume loss were present in the most severely affected patients. The corpus callosum was abnormally thin in all patients. The state of myelination of the CNS was assessed on T2-weighted images. Decreased or absent myelination within supratentorial white-matter tracts was detected in all four patients 10 months of age or older. Myelination of the brainstem and cerebellum progressed normally. No correlation was found between the degree of volume loss or abnormality of myelination demonstrated by MR and the concentration of glycine in the CSF or plasma. Abnormalities shown by MR correlate well with known pathologic findings in patients with nonketotic hyperglycinemia.
Authors:
G A Press; B A Barshop; R H Haas; W L Nyhan; R F Glass; J R Hesselink
Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  AJNR. American journal of neuroradiology     Volume:  10     ISSN:  0195-6108     ISO Abbreviation:  AJNR Am J Neuroradiol     Publication Date:    1989 Mar-Apr
Date Detail:
Created Date:  1989-04-24     Completed Date:  1989-04-24     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:  315-21     Citation Subset:  IM    
Affiliation:
Department of Radiology, University of California, School of Medicine, San Diego 92103.
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Metabolism, Inborn Errors / diagnosis,  genetics*
Atrophy
Brain / pathology*
Child, Preschool
Demyelinating Diseases / diagnosis,  genetics
Female
Glycine / blood*
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging*
Male
Grant Support
ID/Acronym/Agency:
HD04608/HD/NICHD NIH HHS; NS22343/NS/NINDS NIH HHS; R00827//PHS HHS
Chemical
Reg. No./Substance:
56-40-6/Glycine

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


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