Adult-onset chorea and dementia with propionic acidemia.
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.
Sethi
K D
KD
Department of Neurology, Medical College of Georgia, Augusta.
Ray
R
R
Roesel
R A
RA
Carter
A L
AL
Gallagher
B B
BB
Loring
D W
DW
Hommes
F A
FA
D000328:Adult; D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications*; D002819:Chorea / Q000209:etiology*; D003704:Dementia / Q000209:etiology*; D006801:Humans; D008297:Male; D011422:Propionates / Q000097:blood* / Q000378:metabolism
D011422:Propionates