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Case Reports
. 1988 Dec;113(6):1022-7.
doi: 10.1016/s0022-3476(88)80574-2.

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

R Heidenreich  1 , M Natowicz, B E Hainline, P Berman, R I Kelley, R E Hillman, G T Berry
Affiliations
Free article
Case Reports

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

R Heidenreich et al. J Pediatr. 1988 Dec.
Free article

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 or 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.

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Comment in

  • Metabolic stroke in methylmalonic acidemia.
    Thompson GN, Christodoulou J, Danks DM. Thompson GN, et al. J Pediatr. 1989 Sep;115(3):499-500. doi: 10.1016/s0022-3476(89)80867-4. J Pediatr. 1989. PMID: 2597263 No abstract available.

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