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NEUROLOGY 1989;39:1343
© 1989 American Academy of Neurology

Adult-onset chorea and dementia with propionic acidemia

K. D. Sethi, MD, MRCP, R. Ray, MD, R. A. Roesel, PhD, A. L. Carter, PhD, B. B. Gallagher, MD, PhD, D. W. Loring, PhD and F. A. Hommes, PhD

Department of Neurology, Medical College of Georgia, Augusta, GA. (Drs. Sethi, Ray. Gallagher, and Loring)
Cell and Molecular Biology, Medical College of Georgia, Augusta, GA. (Drs. Roesel, Carter, and Hommes)

Propionic acidemia usually presents in the newborn period with severe metabolic acidosis and lethargy. A 31-year-old man with adult onset chorea and dementia had propionic acidemia due to propionyl CoA carboxylase deficiency. Metabolic investigations may prove useful in patients with movement disorder of unknown etiology.

Address correspondence and reprint requests to Dr. Sethi, Department of Neurology, BIW 340, Medical College of Georgia, Augusta, GA 30912.

Presented at the thirty-ninth annual meeting of the American Academy of Neurology, New York, NY, April 1987.

Received February 1, 1989. Accepted for publication in final form April 18, 1989.




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Arch Neurol, September 1, 1999; 56(9): 1143 - 1147.
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