Wernicke-like encephalopathy during classic maple syrup urine disease decompensation.
J Inherit Metab Dis
; 35(3): 413-7, 2012 May.
Article
em En
| MEDLINE
| ID: mdl-22350544
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Encefalopatia de Wernicke
/
Doença da Urina de Xarope de Bordo
Tipo de estudo:
Prognostic_studies
Limite:
Child
/
Female
/
Humans
/
Male
País como assunto:
Europa
Idioma:
En
Ano de publicação:
2012
Tipo de documento:
Article