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Abstract #2214

Brain Metabolites in Myotonic Dystrophy Type 1: A 3.0 T Proton Magnetic Resonance Spectroscopy Study

Yuhei Takado1,2, Hironaka Igarashi1, Kenshi Terajima1,2, Takayoshi Shimohata2, Masaki Okubo1,3, Kouichirou Okamoto1,4, Masatoyo Nishizawa1,2, Tsutomu Nakada1

1Center for Integrated Human Brain Science, Brain Research Institute, University of Niigata, Niigata, Japan; 2Neurology, Brain Research Institute, University of Niigata, Niigata, Japan; 3Institute of Medicine & Dentistry Basic Radiological Technology, School of Health Sciences, University of Niigata, Niigata, Japan; 4Neurosurgery, Brain Research Institute, University of Niigata, Niigata, Japan


The purpose of this study is to seek cerebral abnormalities in myotonic dystrophy type1 (DM1) using 1H-MRS at a 3.0 T scanner. We performed Single-voxel 1H-MRS (frontal white matter and frontal cortex) and MRSI. Brain metabolites were correlated with frontal assessment battery (FAB). The ratio of NAA/Cr in the frontal cortex was negatively correlated with the number of trinucleotide cytosine-thymine-guanine repeats (r = -0.67, p < 0.05). The ratio of NAA/Cr in the frontal white matter was positively correlated with FAB (r = 0.62, p < 0.05). These results probably reflect the microenvironment of the CNS impairment in DM1 patients.