National Yang-Ming University School of Medicine, Taipei, Taiwan.
PLoS One. 2012;7(10):e47925. doi: 10.1371/journal.pone.0047925. Epub 2012 Oct 31.
A broad spectrum of diseases can manifest cerebellar ataxia. In this study, we investigated whether proton magnetic resonance spectroscopy (MRS) may help differentiate spinocerebellar ataxias (SCA) from multiple systemic atrophy- cerebellar type (MSA-C).
This prospective study recruited 156 patients with ataxia, including spinocerebellar ataxia (SCA) types 1, 2, 3, 6 and 17 (N = 94) and MSA-C (N = 62), and 44 healthy controls. Single voxel proton MRS in the cerebellar hemispheres and vermis were measured. The differences were evaluated using nonparametric statistic tests.
When compared with healthy controls, the cerebellar and vermis NAA/Cr and NAA/Cho were lower in all patients(p<0.002). The Cho/Cr was lower in SCA2 and MSA-C (p<0.0005). The NAA/Cr and Cho/Cr were lower in MSA-C or SCA2 comparing with SCA3 or SCA6. The MRS features of SCA1 were in between (p<0.018). The cerebellar NAA/Cho was lower in SCA2 than SCA1, SCA3 or SCA6 (p<0.04). The cerebellar NAA/Cho in MSA-C was lower than SCA3 (p<0.0005). In the early stages of diseases (SARA score<10), significant lower NAA/Cr and NAA/Cho in SCA2, SCA3, SCA6 or MSA-C were observed comparing with healthy controls (p<0.017). The Cho/Cr was lower in MSA-C or SCA2 (p<0.0005). Patients with MSA-C and SCA2 had lower NAA/Cr and Cho/Cr than SCA3 or SCA6 (p<0.016).
By using MRS, significantly lower NAA/Cr, Cho/Cr and NAA/Cho in the cerebellar hemispheres and vermis were found in patients with ataxia (SCAs and MSA-C). Rapid neuronal degeneration and impairment of membrane activities were observed more often in patients with MSA-C than those with SCA, even in early stages. MRS could also help distinguish between SCA2 and other subtypes of SCAs. MRS ratios may be of use as biomarkers in early stages of disease and should be further assessed in a longitudinal study.
许多疾病都会表现出小脑共济失调。本研究旨在探讨质子磁共振波谱（MRS）是否有助于区分脊髓小脑共济失调（SCA）和多系统萎缩-小脑型（MSA-C）。
本前瞻性研究纳入了 156 例共济失调患者，包括 SCA 1、2、3、6 和 17 型（N=94）和 MSA-C（N=62），以及 44 例健康对照者。对小脑半球和蚓部进行单体素质子 MRS 测量。采用非参数统计检验评估差异。
与健康对照组相比，所有患者的小脑和蚓部 NAA/Cr 和 NAA/Cho 均降低（p<0.002）。SCA2 和 MSA-C 的 Cho/Cr 降低（p<0.0005）。与 SCA3 或 SCA6 相比，MSA-C 或 SCA2 的 NAA/Cr 和 Cho/Cr 降低。SCA1 的 MRS 特征介于两者之间（p<0.018）。SCA2 的小脑 NAA/Cho 低于 SCA1、SCA3 或 SCA6（p<0.04）。MSA-C 的小脑 NAA/Cho 低于 SCA3（p<0.0005）。在疾病的早期阶段（SARA 评分<10），与健康对照组相比，SCA2、SCA3、SCA6 或 MSA-C 的 NAA/Cr 和 NAA/Cho 显著降低（p<0.017）。Cho/Cr 降低见于 MSA-C 或 SCA2（p<0.0005）。与 SCA3 或 SCA6 相比，MSA-C 和 SCA2 患者的 NAA/Cr 和 Cho/Cr 降低（p<0.016）。
通过 MRS，发现共济失调患者（SCA 和 MSA-C）的小脑半球和蚓部的 NAA/Cr、Cho/Cr 和 NAA/Cho 明显降低。与 SCA 相比，MSA-C 患者的神经元更快变性和膜活性受损，甚至在疾病早期阶段也是如此。MRS 还可以帮助区分 SCA2 和其他 SCA 亚型。MRS 比值可作为疾病早期阶段的生物标志物，并应在纵向研究中进一步评估。
