Wernicke-like encephalopathy during classic maple syrup urine disease decompensation期刊界 All Journals 搜尽天下杂志传播学术成果专业期刊搜索期刊信息化学术搜索

Wernicke-like encephalopathy during classic maple syrup urine disease decompensation

Authors:	R.?Manara,M.?Del?Rizzo,A.?P.?Burlina,A.?Bordugo,V.?Citton,P.?Rodriguez-Pombo,M.?Ugarte,A.?B.?Burlina author-information" > author-information__contact u-icon-before" > mailto:alberto.burlina@unipd.it" title=" alberto.burlina@unipd.it" itemprop=" email" data-track=" click" data-track-action=" Email author" data-track-label=" " >Email author

Affiliation:	1.Division of Neuroradiology,University Hospital of Padua,Padua,Italy;2.Division of Metabolic Diseases, Department of Paediatrics,University Hospital of Padua,Padua,Italy;3.Division of Neurology,St. Bassiano Hospital,Bassano del Grappa,Italy;4.Centro de Diagnóstico de Enfermedades Moleculares, Centro de Biología Molecular “Severo Ochoa” CSIC-UAM, Departamento de Biología Molecular,Universidad Autónoma de Madrid,Madrid,Spain

Abstract:	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 aware of MSUD as a possible predisposing factor of WE in children.

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