Objective To measure cortical metabolite deficits in vivo in syndromes associated with frontotemporal lobar degeneration, in relation to cognitive and behavioral change. Methods Sixty patients were recruited with a clinical syndrome associated with frontotemporal lobar degeneration (behavioral variant frontotemporal dementia n=11, progressive supranuclear palsy n=26, corticobasal syndrome n=11, primary progressive aphasias n=12), and 38 age- and sex-matched healthy controls. We measured nine metabolites in the right inferior frontal gyrus, superior temporal gyrus and right primary visual cortex using 3T semi-laser magnetic resonance spectroscopy. Metabolite concentrations were corrected for age, sex, and partial volume. We related corrected metabolite concentrations to cognitive and behavioral measures using canonical correlation analysis. Results Metabolite concentrations varied significantly by brain region and diagnosis (region x metabolite x diagnosis interaction F(64)=1.73, p<0.001, corrected for age, sex, and atrophy within the voxel). N-acetyl aspartate and glutamate concentrations were reduced in the right prefrontal cortex in behavioral variant frontotemporal dementia and progressive supranuclear palsy, even after partial volume correction. The reduction of these metabolites was associated with executive dysfunction and behavioral impairment (canonical correlation analysis R=0.95, p<0.001). Conclusion Magnetic resonance spectroscopy confirms behaviourally relevant metabolite deficits including glutamate, in syndromes associated with frontotemporal lobar degeneration. Magnetic resonance spectroscopy may be a useful index of neurodegeneration, and highlight candidates for pharmacological treatment.

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额颞叶变性相关综合征的质子磁共振波谱

目的探讨与额颞叶变性相关的综合征的体内皮质代谢物缺陷，与认知和行为变化有关。方法招募了60例患有额颞叶变性的临床综合征（行为变异型额颞痴呆n = 11，进行性核上性麻痹n = 26，皮质基底综合征n = 11，原发性进行性失语症n = 12），年龄和性别均为38岁匹配的健康对照。我们使用3T半激光磁共振波谱仪测量了右下额回，颞上回和右主视皮层中的9种代谢物。对年龄，性别和部分体积的代谢物浓度进行校正。我们使用规范的相关性分析将校正后的代谢物浓度与认知和行为指标相关联。结果代谢物浓度随大脑区域和诊断的不同而有显着差异（区域x代谢物x诊断相互作用F（64）= 1.73，p <0.001，已针对体素中的年龄，性别和萎缩校正）。行为变异的额颞痴呆症和进行性核上性麻痹的右额叶前皮质中N-乙酰天门冬氨酸和谷氨酸的浓度降低，即使经过部分体积校正也是如此。这些代谢物的减少与执行功能障碍和行为障碍有关（规范相关分析，R = 0.95，p <0.001）。结论磁共振波谱证实与额颞叶变性相关的综合征中与行为有关的代谢产物缺陷，包括谷氨酸。磁共振波谱可能是神经变性的有用指标，