Acute basal ganglia infarction in propionic acidemia

R. H. Haas, D. L. Marsden, S. Capistrano-Estrada, R. Hamilton, Marjorie Grafe, W. Wong, W. L. Nyhan

Research output: Contribution to journalArticle

48 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)18-22
Number of pages5
JournalJournal of Child Neurology
Volume10
Issue number1
StatePublished - 1995
Externally publishedYes

Fingerprint

Propionic Acidemia
Basal Ganglia
Infarction
Muscle Hypotonia
Globus Pallidus
Acids
Putamen
Aphasia
Muscle Weakness
Cerebrospinal Fluid
Necrosis
Stroke
Urine

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Haas, R. H., Marsden, D. L., Capistrano-Estrada, S., Hamilton, R., Grafe, M., Wong, W., & Nyhan, W. L. (1995). Acute basal ganglia infarction in propionic acidemia. Journal of Child Neurology, 10(1), 18-22.

Acute basal ganglia infarction in propionic acidemia. / Haas, R. H.; Marsden, D. L.; Capistrano-Estrada, S.; Hamilton, R.; Grafe, Marjorie; Wong, W.; Nyhan, W. L.

In: Journal of Child Neurology, Vol. 10, No. 1, 1995, p. 18-22.

Research output: Contribution to journalArticle

Haas, RH, Marsden, DL, Capistrano-Estrada, S, Hamilton, R, Grafe, M, Wong, W & Nyhan, WL 1995, 'Acute basal ganglia infarction in propionic acidemia', Journal of Child Neurology, vol. 10, no. 1, pp. 18-22.
Haas RH, Marsden DL, Capistrano-Estrada S, Hamilton R, Grafe M, Wong W et al. Acute basal ganglia infarction in propionic acidemia. Journal of Child Neurology. 1995;10(1):18-22.
Haas, R. H. ; Marsden, D. L. ; Capistrano-Estrada, S. ; Hamilton, R. ; Grafe, Marjorie ; Wong, W. ; Nyhan, W. L. / Acute basal ganglia infarction in propionic acidemia. In: Journal of Child Neurology. 1995 ; Vol. 10, No. 1. pp. 18-22.
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