Wernicke-like encephalopathy during classic maple syrup urine disease decompensation


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… (More)
DOI: 10.1007/s10545-012-9456-3


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