Jun-Ichi Takanashi1,2, Shigeyoshi Saito1, Ichio Aoki1, A. James Barkovich3, Hitoshi Terada4, Yukiko Ito5, Ken Inoue5
1Molecular Imaging Center, National Institute of Radiological Sciences, Chiba, Japan; 2Pediatrics, Kameda Medical Center, Kamogawa, Chiba, Japan; 3Radiology & Biomedical Imaging, University of California Sanfrancisco, San Francisco, CA, United States; 4Radiology, Toho University Sakura Medical Center, Sakura, Chiba, Japan; 5Mental Retardation & Birth Defect Research, National Center of Neurology & Psychiatry, Kodaira, Tokyo, Japan
To evaluate a hypothesis that hypomyelinating process may affect N-acetylaspartate (NAA) and N-acetylaspartylglutamate (NAAG) biochemical pathways, we performed single voxel 1H-MRS for msd mice (model of Pelizaeus-Merzbacher disease) with a 7.0 tesla magnet. 1H-MRS in msd mice revealed increased tNAA (NAA+NAAG) and decreased choline. HPLC analysis revealed increases of both in msd brain. This study suggested hypomyelination could affect NAA and NAAG biochemical pathways leading to increase both of them. Increased tNAA with decreased choline on 1H-MRS may be an important marker for hypomyelinating disorders, which can be distinguished from more common neurological disorders with decreased tNAA.