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

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

110 Scopus citations


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
JournalThe Journal of pediatrics
Issue number6
StatePublished - Dec 1988


ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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