This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

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).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Recurrent Muscle Weakness and Ataxia in Thiamine-Responsive Pyruvate Dehydrogenase Complex Deficiency
Kinoshita et al.
J Child Neurol 1997;12:141-144.
 

Leigh Syndrome: Pyruvate Dehydrogenase Defect. A Case With Peripheral Neuropathy
Chabrol et al.
J Child Neurol 1994;9:52-55.
ABSTRACT  

Topical Review Article: Organic Acidurias: A Review. Part 1
Ozand and Gascon
J Child Neurol 1991;6:196-219.
ABSTRACT  

Magnetic Resonance Imaging in a Case of Autopsy-Proved Adult Subacute Necrotizing Encephalomyelopathy (Leigh's Disease)
Kissel et al.
Arch Neurol 1987;44:563-566.
ABSTRACT