Document Detail

Methylmalonic and malonic aciduria in a dog with progressive encephalomyelopathy.
MedLine Citation:
PMID:  8869944     Owner:  NLM     Status:  MEDLINE    
A 12 week old female Labrador retriever dog with signs of progressive diffuse degeneration of the brain and spinal cord was found to have methylmalonic and malonic aciduria. Over a 5 month period, the dog developed neurologic signs compatible with disease of the central nervous system with predominant diffuse cerebral and right lateralizing brainstem deficits. Gross pathological examination of the brain showed that the lateral, third, and fourth ventricles of the brain were markedly enlarged and associated with white and grey matter atrophy. Syringomyelia and hydromyelia of the central canal into the dorsal funiculus of the spinal cord beginning at the level of the cervical intumescence and extending to the lumbar intumescence was also present. Significant biochemical abnormalities include methylmalonic and malonic aciduria, mild lactic and pyruvic aciduria. There was also accumulation of citric acid cycle intermediates including succinic, aconitic, and fumaric acids. Disordered fatty acid oxidation was suggested by increased excretion of adipic, ethylmalonic, suberic and sebacic acids. Neither ketoacidosis nor hyperammonemia were present, and serum cobalamin levels were normal. Overall, this dog demonstrates an inborn error of metabolism resulting in abnormal organic acid accumulation associated with a neurodegenerative disease.
M Podell; G D Shelton; W L Nyhan; S O Wagner; A Genders; M Oglesbee; W R Fenner
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Metabolic brain disease     Volume:  11     ISSN:  0885-7490     ISO Abbreviation:  Metab Brain Dis     Publication Date:  1996 Sep 
Date Detail:
Created Date:  1997-01-23     Completed Date:  1997-01-23     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8610370     Medline TA:  Metab Brain Dis     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  239-47     Citation Subset:  IM    
Department of Veterinary Clinical Sciences, Ohio State University, Columbus 43210, USA.
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MeSH Terms
Brain / pathology,  ultrastructure
Dog Diseases / pathology*,  urine*
Encephalomyelitis / pathology,  urine*,  veterinary*
Gas Chromatography-Mass Spectrometry
Hydrocephalus / metabolism,  pathology
Malonates / urine*
Metabolism, Inborn Errors / pathology,  urine*,  veterinary*
Methylmalonic Acid / urine*
Muscles / innervation,  pathology,  ultrastructure
Nerve Fibers / ultrastructure
Reg. No./Substance:
0/Malonates; 141-82-2/malonic acid; 516-05-2/Methylmalonic Acid

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

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