Wernicke-like encephalopathy during classic maple syrup urine disease decompensation

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

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

We describe a new neuroradiologic picture observed during metabolic decompensation in two maple syrup urine disease (MSUD) patients that resemblesWernicke 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.

Original languageEnglish
Pages (from-to)413-417
Number of pages5
JournalJournal of Inherited Metabolic Disease
Volume35
Issue number3
DOIs
Publication statusPublished - May 2012

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Maple Syrup Urine Disease
Wernicke Encephalopathy
Brain Diseases
Causality

ASJC Scopus subject areas

  • Genetics(clinical)
  • Genetics

Cite this

Manara, R., Del Rizzo, M., Burlina, A. P., Bordugo, A., Citton, V., Rodriguez-Pombo, P., ... Burlina, A. B. (2012). Wernicke-like encephalopathy during classic maple syrup urine disease decompensation. Journal of Inherited Metabolic Disease, 35(3), 413-417. https://doi.org/10.1007/s10545-012-9456-3

Wernicke-like encephalopathy during classic maple syrup urine disease decompensation. / Manara, R.; Del Rizzo, M.; Burlina, A. P.; Bordugo, A.; Citton, V.; Rodriguez-Pombo, P.; Ugarte, M.; Burlina, A. B.

In: Journal of Inherited Metabolic Disease, Vol. 35, No. 3, 05.2012, p. 413-417.

Research output: Contribution to journalArticle

Manara, R, Del Rizzo, M, Burlina, AP, Bordugo, A, Citton, V, Rodriguez-Pombo, P, Ugarte, M & Burlina, AB 2012, 'Wernicke-like encephalopathy during classic maple syrup urine disease decompensation', Journal of Inherited Metabolic Disease, vol. 35, no. 3, pp. 413-417. https://doi.org/10.1007/s10545-012-9456-3
Manara R, Del Rizzo M, Burlina AP, Bordugo A, Citton V, Rodriguez-Pombo P et al. Wernicke-like encephalopathy during classic maple syrup urine disease decompensation. Journal of Inherited Metabolic Disease. 2012 May;35(3):413-417. https://doi.org/10.1007/s10545-012-9456-3
Manara, R. ; Del Rizzo, M. ; Burlina, A. P. ; Bordugo, A. ; Citton, V. ; Rodriguez-Pombo, P. ; Ugarte, M. ; Burlina, A. B. / Wernicke-like encephalopathy during classic maple syrup urine disease decompensation. In: Journal of Inherited Metabolic Disease. 2012 ; Vol. 35, No. 3. pp. 413-417.
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