Congenital muscular dystrophy with merosin deficiency:: 1H MR spectroscopy and diffusion-weighted MR imaging
Congenital muscular dystrophy with merosin deficiency:: 1H MR spectroscopy and diffusion-weighted MR imaging
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DOI：
10.1148/radiol.2351031963
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发表时间：
2005-04-01
期刊：
影响因子：
19.7
通讯作者：
Cerri, GG
中科院分区：
文献类型：
--
作者：
Leite, CC;Reed, UC;Cerri, GG
目的：前瞻性地使用氢 1 (H-1) 磁共振 (MR) 波谱和表观扩散系数 (ADC) 图来尝试解释 MR 成像中观察到的广泛白质 (WM) 异常与 Merosin 缺陷型先天性肌营养不良 (CMD) 患者相对轻微的神经认知能力下降之间的差异。 材料和方法：医院伦理 委员会批准了这项研究，并获得了知情同意。 9 名患有 Merosin 缺乏 CMD 的患者（5 名男孩，4 名女孩；年龄范围 3-9 岁；平均 6 岁 +/- 2 [标准差]）接受了 T1 加权、T2 加权、液体衰减反转恢复、扩散加权 MR 成像和 H-1 MR 波谱，在顶枕 WM (POWM) 和额叶 WM 中进行 （FWM）通过使用受激回波采集模式。计算代谢物（N-乙酰天冬氨酸 [NAA]、含胆碱化合物 [Cho] 和肌醇 [ml]）与肌酸/磷酸肌酸 (Cr) 和水 (H2O) 的关系。还计算了 NAA/Cho。 ADC 的计算位置与光谱学研究的位置大致相同。为了进行比较，还在 10 名健康年龄和性别匹配的对照受试者（三名男孩，七名女孩；年龄范围，4-9 岁；平均，6 岁+/- 1）中进行了 H-1 MR 波谱 (n = 10) 和 ADC 映射 (n = 7)。统计分析涉及不同组间比较的t检验；通过 Pearson 检验研究了 ADC 和光谱结果之间的相关性。结果：MR 成像显示所有患者双侧 WM 受累的证据。虽然他们的 NAA/Cr 和 Cho/Cr 正常，但与对照受试者相比，他们的 ml/Cr 略有增加（FWM 中 P =.03，POWM 中 P =.07），而 POWM 中他们的 NAA/Cho 降低（P =.03）。 NAA/H2O、Cr/H2O、Cho/H2O 和 ml/H2O 显着降低（P
PURPOSE: To prospectively use hydrogen 1 (H-1) magnetic resonance (MR) spectroscopy and apparent diffusion coefficient (ADC) maps to try to explain the discrepancy between the extensive white matter (WM) abnormalities observed at MR imaging and the relatively mild neurocognitive decline in patients with merosin-deficient congenital muscular dystrophy (CMD).MATERIALS AND METHODS: The hospital ethics committee approved this study, and informed consent was obtained. Nine patients (five boys, four girls; age range, 3-9 years; mean, 6 years +/- 2 [standard deviation]) with merosin-deficient CMD underwent T1-weighted, T2-weighted, fluid-attenuated inversion recovery, and diffusion-weighted MR imaging and H-1 MR spectroscopy, which was performed in the parieto-occipital WM (POWM) and frontal WM (FWM) by using stimulated-echo acquisition mode. Metabolite (N-acetylaspartate [NAA], choline-containing compounds [Cho], and myo-inositol [ml]) ratios were calculated in relation to creatine/ phosphocreatine (Cr) and water (H2O). NAA/Cho was also calculated. ADCs were calculated in approximately the same locations that were studied with spectroscopy. For comparison, H-1 MR spectroscopy (n = 10) and ADC mapping (n = 7) were also performed in 10 healthy age- and sex-matched control subjects (three boys, seven girls; age range, 4-9 years; mean, 6 years +/- 1). Statistical analysis involved the t test for comparison between different groups; correlation between ADC and spectroscopy results was studied with the Pearson test.RESULTS: MR imaging revealed evidence of bilateral WM involvement in all patients. Whereas their NAA/Cr and Cho/Cr were normal, their ml/Cr was slightly increased compared with that in control subjects (P =.03 in FWM and P =.07 in POWM), and their NAA/Cho was decreased in POWM (P =.03). NAA/H2O, Cr/H2O Cho/H2O, and ml/H2O were considerably decreased (P