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Article

Wernicke-like encephalopathy during classic maple syrup urine disease decompensation.

Journal of inherited metabolic disease - 1 May 2012

Manara R, Del Rizzo M, Burlina A P, Bordugo A, Citton V, Rodriguez-Pombo P, Ugarte M, Burlina A B

Abstract excerpt

We describe a new neuroradiologic picture observed during metabolic decompensation in two maple syrup urine disease (MSUD) patients that resembles Wernicke encephalopathy (WE). Clinical observations and the review of the literature regarding WE and MSUD pathophysiology prompted us to hypothesize a pathogenic link between these two disorders. Based on these findings, clinicians and neuroradiologists should be...

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PMID: 22350544
DOI: 10.1007/s10545-012-9456-3

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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