Diffuse Brain Abnormalities in Myotonic Dystrophy Type 1 Detected by 3.0 T Proton Magnetic Resonance Spectroscopy

被引：13
|
作者：
Takado, Yuhei [1 ,2 ]
Terajima, Kenshi [1 ,2 ,5 ]
Ohkubo, Masaki [1 ,4 ]
Okamoto, Kouichirou [1 ,3 ]
Shimohata, Takayoshi [2 ]
Nishizawa, Masatoyo [1 ,2 ]
Igarashi, Hironaka [1 ]
Nakada, Tsutomu [1 ,6 ]
机构：
[1] Niigata Univ, Ctr Integrated Human Brain Sci, Niigata 9518585, Japan
[2] Niigata Univ, Dept Neurol, Niigata 9518585, Japan
[3] Niigata Univ, Brain Res Inst, Dept Neurosurg, Niigata 9518585, Japan
[4] Niigata Univ, Grad Sch Hlth Sci, Niigata 9518585, Japan
[5] Niigata Univ, Med & Dent Hosp, Dept Med Informat, Niigata 9518585, Japan
[6] Univ Calif Davis, Dept Neurol, Davis, CA 95616 USA
来源：
EUROPEAN NEUROLOGY | 2015年 / 73卷 / 3-4期
关键词：
Myotonic dystrophy; MRS; Cerebral metabolism; Glutamate; Frontal lobe; FRONTAL ASSESSMENT BATTERY; H-1 MR SPECTROSCOPY; IN-VIVO; METABOLITE CONCENTRATIONS; N-ACETYLASPARTATE; CTG REPEATS; DISORDERS; EXPANSION; RAT; QUANTIFICATION;
D O I：
10.1159/000371575
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
Patients with myotonic dystrophy type 1 (DM1) (n = 14) were compared with healthy controls (n = 13) using 3.0 T proton magnetic resonance spectroscopy (H-1-MRS) to investigate brain pathophysiology. H-1-MRS imaging revealed reduced N-acetylaspartate to creatine ratio (NAA/Cr) in multiple brain regions (average 24%), suggesting diffuse brain abnormalities among patients with DM1. Single-voxel H-1-MRS among patients with DM1 showed (1) reduced NAA in both the frontal cortex (23%) and frontal white matter (31%) and unaltered myo-inositol, suggesting neuronal abnormalities without significant gliosis; and (2) elevated glutamine in the frontal cortex (36%) and reduced glutamate in the frontal white matter (20%) among patients with DM1, suggesting abnormalities in the glutamatergic system in the brain of patients with DM1. We consider that these results reflect brain abnormalities that cannot be detected by neuropathological assessment in patients with DM1. (C) 2015 S. Karger AG, Basel
引用
收藏
页码：247 / 256
页数：10
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