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Neurochemistry of Hypomyelination Investigated with MR Spectroscopy
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- TAKANASHI Jun-ichi
- Department of Pediatrics, Tokyo Women's Medical University, Yachiyo Medical Center Molecular Imaging Center, National Institute of Radiological Sciences
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Description
Proton magnetic resonance spectroscopy (MRS) allows the noninvasive exploration of tissue metabolism in vivo, providing neurophysiological and neurochemical information. N-acetylaspartate (NAA) is generally considered to be a marker of neurons and axons, and many neurodegenerative disorders, including demyelinating disorders, exhibit a decrease in total NAA (tNAA). MRS in human hypomyelination disorders, such as Pelizaeus-Merzbacher disease (PMD), is characterized by normal to elevated tNAA, elevated myo-inositol and creatine (Cr), and normal to decreased choline (Cho).<br/>MRS in the thalamus of a hypomyelinating mouse model, a myelin synthesis-deficient (msd) mouse, a model of connatal PMD with mutation of the Plp1 gene, revealed increased tNAA and Cr and decreased Cho. That of a shiverer mouse with an autosomal recessive mutation of the Mbp gene showed decreased Cho with normal tNAA and Cr. Accordingly, the reduction of Cho on MRS might be a common marker for hypomyelinating disorders. tNAA concentrations range from normal to increased, probably depending upon the underlying pathology of oligodendrocytes. tNAA may be increased in hypomyelination with a reduced number of mature oligodendrocytes, such as PMD.
Journal
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- Magnetic Resonance in Medical Sciences
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Magnetic Resonance in Medical Sciences 14 (2), 85-91, 2015
Japanese Society for Magnetic Resonance in Medicine