Proton magnetic resonance spectroscopic imaging in the clinical evaluation of patients with Niemann-Pick type C disease
Proton magnetic resonance spectroscopic imaging in the clinical evaluation of patients with Niemann-Pick type C disease
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DOI：
10.1136/jnnp.65.1.72
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发表时间：
1998-07-01
影响因子：
11
通讯作者：
Schiffmann, R
中科院分区：
文献类型：
--
作者：
Tedeschi, G;Bonavita, S;Schiffmann, R
目的：对10例C型尼曼匹克病（NP-C）患者进行质子磁共振波谱成像研究方法-H-1-MRSI允许同时测量N-乙酰天冬氨酸（NA）、含胆碱的化合物（Cho）、肌酸加磷酸肌酸（Cre）、和乳酸（Lac）信号强度，来自分成0.84 ml单个体积单元的四个15 mm切片。光谱体素被确定从七个区域的interests.Results-In患者NP-C，NA/Cre显着下降，在额叶和顶叶皮质，半卵圆中心，尾状核; Cho/Cre显着增加，在额叶皮质和半卵圆中心。临床分期量表评分与H-1-MRSI异常之间存在显著相关性。结论-H-1-MRSI显示NP-C患者弥漫性脑受累与疾病的病理特征一致。H-1-MRSI是一个客观和敏感的工具，神经系统评估与NP-C患者。
Objectives-10 patients with Niemann-Pick disease type C (NP-C) were studied by proton magnetic resonance spectroscopic imaging (H-1-MRSI) to assess the biochemical pathology of the brain and to determine whether this method can be useful to clinically evaluate these patients.Methods-H-1-MRSI permits the simultaneous measurement of N-acetyl aspartate (NA), compounds containing choline (Cho), creatine plus phosphocreatine (Cre), and lactate (Lac) signal intensities from four 15 mm slices divided into 0.84 mi single volume elements. Spectroscopic voxels were identified from seven regions of interest.Results-In patients with NP-C, NA/Cre was significantly decreased in the frontal and parietal cortices, centrum semiovale, and caudate nucleus; Cho/Cre was significantly increased in the frontal cortex and centrum semiovale. Significant correlations were found between clinical staging scale scores and H-1-MRSI abnormalities.Conclusion-H-1-MRSI showed diffuse brain involvement in patients with NP-C consistent with the pathological features of the disease. H-1-MRSI is an objective and sensitive tool to neurologically evaluate patients with NP-C.