Document Detail

Pyruvate decarboxylase deficiency in subacute necrotizing encephalomyelopathy.
MedLine Citation:
PMID:  7247788     Owner:  NLM     Status:  MEDLINE    
A partial deficiency of pyruvate decarboxylase (PDC) was demonstrated in a child with hyperlactatemia and progressive ataxia, bulbar paresis, ophthalmoplegia, and polyneuropathy. Subacute necrotizing encephalomyelopathy (SNE) was found at necropsy. The association of SNE and PDC deficiency has been reported rarely, but a review of the diverse metabolic defects associated with SNE suggests that decreased PDC activity may be the common feature of SNE.
O B Evans
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of neurology     Volume:  38     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  1981 Aug 
Date Detail:
Created Date:  1981-08-27     Completed Date:  1981-08-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  515-9     Citation Subset:  AIM; IM    
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MeSH Terms
Alanine / blood
Ataxia / diagnosis
Brain / pathology
Brain Diseases / diagnosis,  metabolism*,  pathology
Carbohydrate Metabolism, Inborn Errors / diagnosis
Carboxy-Lyases / deficiency*
Lactates / blood
Paralysis / diagnosis
Pyruvate Decarboxylase / deficiency*
Pyruvates / blood
Reg. No./Substance:
0/Lactates; 0/Pyruvates; 56-41-7/Alanine; EC 4.1.1.-/Carboxy-Lyases; EC Decarboxylase

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

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