Abstract #2214

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

Yuhei Takado^1,2, Hironaka Igarashi¹, Kenshi Terajima^1,2, Takayoshi Shimohata², Masaki Okubo^1,3, Kouichirou Okamoto^1,4, Masatoyo Nishizawa^1,2, 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.