Diffuse brain abnormalities in myotonic dystrophy type 1 detected by 3.0 T proton magnetic resonance spectroscopy
Diffuse brain abnormalities in myotonic dystrophy type 1 detected by 3.0 T proton magnetic resonance spectroscopy
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3.0 T质子磁共振波谱检测强直性肌营养不良1型弥漫性脑异常
作者：
Takado Y;Terajima K;Ohkubo M;Okamoto K;Shimohata T;Nishizawa M;Igarashi H;Nakada T
应用3.0T质子磁共振波谱（1H-MRS）对14例1型强直性肌营养不良（DM 1）患者和13例健康对照者进行脑病理生理学研究，结果显示DM 1患者多个脑区N-乙酰天门冬氨酸/肌酸比值（NAA/Cr）降低（平均24%），提示DM 1患者存在弥漫性脑异常。DM 1患者的单体素1H-MRS显示：（1）额叶皮质（23%）和额叶白色物质（31%）的NAA减少，而肌醇不变，提示神经元异常，但无明显胶质增生;（2）DM 1患者额叶皮质谷氨酰胺升高（36%），额叶白色物质谷氨酸降低（20%），提示DM 1患者大脑中的多巴胺能系统异常。我们认为这些结果反映了DM 1患者的神经病理学评估无法检测到的大脑异常。
Patients with myotonic dystrophy type 1 (DM1) (n = 14) were compared with healthy controls (n = 13) using 3.0 T proton magnetic resonance spectroscopy (1H-MRS) to investigate brain pathophysiology.1H-MRS imaging revealed reducedN-acetylaspartate to creatine ratio (NAA/Cr) in multiple brain regions (average 24%), suggesting diffuse brain abnormalities among patients with DM1. Single-voxel1H-MRS among patients with DM1 showed (1) reduced NAA in both the frontal cortex (23%) and frontal white matter (31%) and unalteredmyo-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.