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Review
. 1990 May;149(8):582-4.
doi: 10.1007/BF01957698.

Symmetrical necrosis of the basal ganglia in methylmalonic acidaemia

A M Roodhooft  1 , E R Baumgartner, J J Martin, W Blom, K J Van Acker
Affiliations
Review

Symmetrical necrosis of the basal ganglia in methylmalonic acidaemia

A M Roodhooft et al. Eur J Pediatr. 1990 May.

Abstract

In a patient with methylmalonic acidaemia (MMAA), persistent neurological symptoms were observed in addition to the acute episodes of metabolic dysequilibrium. CT scan and magnetic resonance imaging revealed bilateral symmetrical necrosis of the globus pallidus. Different episodes of metabolic decompensation, one with severe acidosis, had occurred. Persistent neurological symptoms in patients with MMAA who are appropriately treated suggest irreversible brain damage which appears to occur preferentially at the level of the basal ganglia.

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