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  Vol. 38 No. 8, August 1981 TABLE OF CONTENTS
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Pyruvate Decarboxylase Deficiency in Subacute Necrotizing Encephalomyelopathy

Owen B. Evans, MD

Arch Neurol. 1981;38(8):515-519.


Abstract

• 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.



Author Affiliations

From the Department of Neurology and the Jerry Lewis Neuromuscular Research Center, Vanderbilt University, Nashville, Tenn.


Footnotes

Accepted for publication Sept 19, 1980.

Reprint requests to Department of Neurology and the Jerry Lewis Neuromuscular Research Center, Vanderbilt University School of Medicine, Nashville, TN 37232 (Dr Evans).



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