Acute Basal Ganglia Infarction in Propionic Acidemia

Richard H. Haas, Deborah L. Marsden, Sylvia Capistrano-Estrada, Ronald Hamilton, Marjorie R. Grafe, William L. Nyhan, Wade Wong

Research output: Contribution to journalArticlepeer-review

58 Scopus citations


An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of “metabolic stroke” is more complicated than previously thought. (J Child Neurol 1995;10:18-22).

Original languageEnglish (US)
Pages (from-to)18-22
Number of pages5
JournalJournal of child neurology
Issue number1
StatePublished - Jan 1995
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology


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