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Literature DB >> 22350544

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

R Manara¹, M Del Rizzo, A P Burlina, A Bordugo, V Citton, P Rodriguez-Pombo, M Ugarte, A B Burlina.

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.

Entities: Disease Mutation Species

Mesh：

Year: 2012 PMID： 22350544 DOI： 10.1007/s10545-012-9456-3

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

16 in total

1. Metabolic decompensation and lactic acidosis in propionic acidaemia complicated by thiamine deficiency.

Authors: E Mayatepek; A Schulze
Journal: J Inherit Metab Dis Date: 1999-04 Impact factor: 4.982

2. Metronidazole-induced and Wernicke encephalopathy: two different entities sharing the same metabolic pathway?

Authors: G Zuccoli; N Pipitone; D Santa Cruz
Journal: AJNR Am J Neuroradiol Date: 2008-06-19 Impact factor: 3.825

Review 3. Role of mitochondrial dysfunction and oxidative stress in the pathogenesis of selective neuronal loss in Wernicke's encephalopathy.

Authors: Paul Desjardins; Roger F Butterworth
Journal: Mol Neurobiol Date: 2005 Impact factor: 5.590

Review 4. Effects of thiamine deficiency on brain metabolism: implications for the pathogenesis of the Wernicke-Korsakoff syndrome.

Authors: R F Butterworth
Journal: Alcohol Alcohol Date: 1989 Impact factor: 2.826

Review 5. Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management.

Authors: Gianpietro Sechi; Alessandro Serra
Journal: Lancet Neurol Date: 2007-05 Impact factor: 44.182

6. Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.

Authors: Alexandre Umpierrez Amaral; Guilhian Leipnitz; Carolina Gonçalves Fernandes; Bianca Seminotti; Patrícia Fernanda Schuck; Moacir Wajner
Journal: Brain Res Date: 2010-02-11 Impact factor: 3.252

7. Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain.

Authors: M S Patel
Journal: Biochem J Date: 1974-10 Impact factor: 3.857

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

1. Metabolic decompensation and lactic acidosis in propionic acidaemia complicated by thiamine deficiency.

2. Metronidazole-induced and Wernicke encephalopathy: two different entities sharing the same metabolic pathway?

Review 3. Role of mitochondrial dysfunction and oxidative stress in the pathogenesis of selective neuronal loss in Wernicke's encephalopathy.

Review 4. Effects of thiamine deficiency on brain metabolism: implications for the pathogenesis of the Wernicke-Korsakoff syndrome.

Review 5. Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management.

6. Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.

7. Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain.

8. Clinical signs in the Wernicke-Korsakoff complex: a retrospective analysis of 131 cases diagnosed at necropsy.

Review 9. Pathogenesis of CNS involvement in disorders of amino and organic acid metabolism.

10. Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease.

Review 1. Maple syrup urine disease: mechanisms and management.

2. Maple Syrup Urine Disease Masquerading as Urea Cycle Disorder: A Tale of Two Clinical Mimics.

3. Silico analysis of a novel mutation c.550delT in a Chinese patient with maple syrup urine disease.