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MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation

✍ Scribed by Wajanat Jan; Robert A. Zimmerman; Zhiyue J. Wang; Gerard T. Berry; Paige B. Kaplan; Edward M. Kaye

Publisher: Springer
Year: 2003
Tongue: English
Weight: 282 KB
Volume: 45
Category: Article
ISSN: 0028-3940
DOI: 10.1007/s00234-003-0955-7

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A 7-month-old boy is reported with acute metabolic crisis of maple syrup urine disease. A reversible intramyelinic type of edema was noted by diffusion MRI which completely resolved in 3 months in accordance with good clinical outcome. Proton MR spectroscopy revealed decreased NAA, and presence of m