Maple syrup urine disease: diffusion MRI, and proton MR spectroscopy findings
RN Sener - Computerized Medical Imaging and Graphics, 2007 - Elsevier
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 methyl resonances of branched chain amino
acids at 0.9 ppm, and lactic acid in the initial examination. After 3 months, NAA returned to
normal, and lactic acid disappeared. The methyl resonance of branched chain amino acids …
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 methyl resonances of branched chain amino
acids at 0.9 ppm, and lactic acid in the initial examination. After 3 months, NAA returned to
normal, and lactic acid disappeared. The methyl resonance of branched chain amino acids …