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Diffusion Magnetic Resonance Imaging in Intermediate Form of Maple Syrup Urine Disease
Author(s) -
Sener R. N.
Publication year - 2002
Publication title -
journal of neuroimaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.822
H-Index - 64
eISSN - 1552-6569
pISSN - 1051-2284
DOI - 10.1111/j.1552-6569.2002.tb00146.x
Subject(s) - medicine , magnetic resonance imaging , effective diffusion coefficient , globus pallidus , nuclear magnetic resonance , diffusion , maple syrup urine disease , diffusion mri , parenchyma , pons , maple , anatomy , pathology , nuclear medicine , central nervous system , radiology , chemistry , biology , basal ganglia , biochemistry , physics , thermodynamics , leucine , botany , amino acid
An 8‐year‐old boy with the intermediate variant of maple syrup urine disease is reported. On b = 1000 s/mm 2 (heavily diffusion weighted) images of diffusion magnetic resonance imaging, there was symmetric high signal in the globus pallidus, mesencephalon, dorsal pons, and nucleus dentatus, consistent with restriction of the mobility of water molecules. Apparent diffusion coefficient (ADC) maps revealed low ADC values ranging from 0.42 to 0.56 ?10 −3 mm 2 /s in these regions, compared to those of apparently unaffected regions in the brain parenchyma ranging from 0.63 to 0.97 ?10 −3 mm 2 /s. It is suggested that the areas of increased signal (and low ADC values) are the result of dysmyelination as a reflection of disorganized tissue integrity.