Brainstem neurodegeneration correlates with clinical dysfunction in SCA1 but not in SCA2. A quantitative volumetric, diffusion and proton spectroscopy MR study
Brainstem neurodegeneration correlates with clinical dysfunction in SCA1 but not in SCA2. A quantitative volumetric, diffusion and proton spectroscopy MR study
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DOI：
10.1093/brain/awh201
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发表时间：
2004-08-01
期刊：
影响因子：
14.5
通讯作者：
Mascalchi, M
中科院分区：
文献类型：
--
作者：
Guerrini, L;Lolli, F;Mascalchi, M
磁共振(MR)技术能够在体内测量脊髓小脑型共济失调1型(SCA1)和2型(SCA2)患者的脑干和小脑萎缩，伴随着脑桥和小脑中N-乙酰天冬氨酸(NAA)浓度或NAA/肌酸比值的下降。平均弥散系数(D/BAR)是研究脑部疾病的又一敏感和定量的MR参数。为了探讨SCA1和SCA2的MR特征的差异，并将两者的MR表现与临床表现相关联，我们对16例SCA1患者、12例SCA2患者和20例健康对照组进行了检查。MR检查包括T1加权3D梯度回波序列、右侧小脑半球(齿状区和齿状区)和桥脑的单体素质子波谱，采用PRESS序列和外部参考定量方法，以及(9例SCA1和9例SCA2患者)弥散加权平面回波图像，重建条形图上的(D)。采用遗传性共济失调临床评定量表(IACRS)对患者进行评定。与对照组相比，SCA1和SCA2显著降低(P<0.05)。
Magnetic resonance (MR) techniques enable in vivo measurement of the atrophy of the brainstem and cerebellum in spinocerebellar ataxia type 1 (SCA1) and 2 (SCA2) patients, which is accompanied by a decrease in the concentration of N-acetyl aspartate (NAA) or of the NAA/creatine ratio in the pons and cerebellum. Mean diffusivity ((D) over bar) is emerging as an additional sensitive and quantitative MR parameter to investigate brain diseases. In order to explore differences between the MR features of SCA1 and SCA2 and correlate the MR and clinical findings in the two conditions, we examined 16 SCA1 patients, 12 SCA2 patients and 20 healthy control subjects. The MR protocol included T1-weighted 3D gradient echo sequences, single-voxel proton spectroscopy of the right cerebellar hemisphere (dentate and peridentate region) and of the pons with a PRESS sequence and an external reference quantitation method, and (in nine patients with SCA1 and nine patients with SCA2) diffusion-weighted echo-planar images with reconstruction of the (D) over bar maps. The patients were evaluated with the Inherited Ataxia Clinical Rating Seale (IACRS). Compared with control subjects, the SCA1 and SCA2 patients showed a decrease (P