Acute extrapyramidal syndrome in methylmalonic acidemia: "Metabolic stroke" involving the globus pallidus

Randall Heidenreich, Marvin Natowicz, Bryan Hainline, Peter Berman, Richard I. Kelley, Richard E. Hillman, Gerard T. Berry

Research output: Contribution to journalArticle

106 Citations (Scopus)

Abstract

We report four patients with methylmalonic acidemia who developed acute extrapyramidal disease after metabolic decompensation. The neurologic findings resulted from bilateral destruction of the globus pallidus with variable involvement of the internal capsules. This complication was unrelated to a specific gene defect responsible for methylmalonic acidemia of to cyanocobalamin administration. These lesions constitute a "metabolic stroke," probably because of the accumulation of toxic organic acid metabolites, because they cannot be accounted for by hypoxemia or vascular insufficiency.

Original languageEnglish (US)
Pages (from-to)1022-1027
Number of pages6
JournalJournal of Pediatrics
Volume113
Issue number6
DOIs
StatePublished - 1988
Externally publishedYes

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Globus Pallidus
Stroke
Internal Capsule
Poisons
Acute Disease
Vitamin B 12
Neurologic Manifestations
Blood Vessels
Acids
Genes
Methylmalonic acidemia
Hypoxia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Acute extrapyramidal syndrome in methylmalonic acidemia : "Metabolic stroke" involving the globus pallidus. / Heidenreich, Randall; Natowicz, Marvin; Hainline, Bryan; Berman, Peter; Kelley, Richard I.; Hillman, Richard E.; Berry, Gerard T.

In: Journal of Pediatrics, Vol. 113, No. 6, 1988, p. 1022-1027.

Research output: Contribution to journalArticle

Heidenreich, Randall ; Natowicz, Marvin ; Hainline, Bryan ; Berman, Peter ; Kelley, Richard I. ; Hillman, Richard E. ; Berry, Gerard T. / Acute extrapyramidal syndrome in methylmalonic acidemia : "Metabolic stroke" involving the globus pallidus. In: Journal of Pediatrics. 1988 ; Vol. 113, No. 6. pp. 1022-1027.
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