In vivo evidence of brain galactitol accumulation in an infant with galactosemia and encephalopathy.

Berry, G T; Hunter, J V; Wang, Z; Dreha, S; Mazur, A; Brooks, D G; Ning, C; Zimmerman, R A et al. · J Pediatr · 2001

case_report · Level V

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Abstract

In a newborn infant with galactose-1-phosphate uridyltransferase deficiency and encephalopathy, brain magnetic resonance imaging revealed cytotoxic edema in white matter. Using in vivo proton magnetic resonance spectroscopy, we detected approximately 8 mmol galactitol per kilogram of brain tissue, an amount potentially relevant to the pathogenesis of brain edema.

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