Adult-onset chorea and dementia with propionic acidemia

Neurology. 1989 Oct;39(10):1343-5. doi: 10.1212/wnl.39.10.1343.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amino Acid Metabolism, Inborn Errors / complications*
  • Chorea / etiology*
  • Dementia / etiology*
  • Humans
  • Male
  • Propionates / blood*
  • Propionates / metabolism


  • Propionates