Alternating Hemiplegia as a Major Symptom of Maple Syrup Urine Disease: Case Report/h1> Volume 4 - Issue 2

Natasha R Geisel^1,3, Luisa N Simonsen^2,3, Marlos MMartins^1,3, Marco Orsini*^4,5 and Andreia SS Moreira^1,3,6

Received: April 21, 2018;   Published: May 07,2018

DOI: 10.26717/BJSTR.2018.04.001034

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Abstract

The aim of the present study is to draw attention to the rare association of maple syrup urine disease and alternating hemiplegia.We present a case of a 3-year-old boy adopted 3 months before the onset of alternating hemiplegia events, with development delay, had investigation with MRI and Urinary organic acid suggestive of MSUD. Started the treatment restriction of leucine, isoleucine and valine intake and thiamine supplementation, with resolution of his neurology symptoms.

Keywords: Hemiplegia; Maple Syrup Urine Disease; Branched Chain Amino Acid; Leucinosis; Paroxysmal

Abstract| Introduction| Case Report| Discussion| Conclusion| References|