Distinct neurochemical profiles of spinocerebellar ataxias 1, 2, 6, and cerebellar multiple system atrophy.
Distinct neurochemical profiles of spinocerebellar ataxias 1, 2, 6, and cerebellar multiple system atrophy.
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DOI：
10.1007/s12311-010-0213-6
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发表时间：
2011-06
期刊：
影响因子：
3.5
通讯作者：
Gomez, Christopher M.
中科院分区：
文献类型：
--
作者：
Oez, Guelin;Iltis, Isabelle;Hutter, Diane;Thomas, William;Bushara, Khalaf O.;Gomez, Christopher M.
遗传性和散发性神经退行性共济失调是影响小脑的运动障碍。稳健和客观的生物标志物对于共济失调的治疗试验至关重要。此外，这些生物标记物可能有助于区分共济失调亚型，因为这些疾病在临床表现和常规MRI上有很大的重叠。通过高场质子磁共振波谱(1HMRS)获得的体内10-13个神经化学浓度的分布可以潜在地提供共济失调类型的特异性生物标记物。我们比较了26例脊髓小脑性共济失调(SCA1，N=9；SCA2，N=7；SCA6，N=5)或小脑多系统萎缩(MSA-C，N=5)患者和15名年龄匹配的健康对照组在4T时测量的小脑和脑干神经化学特征。共济失调评定量表(SARA)用于评估病情严重程度。与对照组相比，不同类型共济失调患者的神经化学改变模式不同。两组患者小脑半球的肌醇、肌醇、总N-乙酰天冬氨酸、总肌酸、谷氨酸、谷氨酰胺和脑桥的肌醇、总N-乙酰天冬氨酸、谷氨酸水平差异有统计学意义(Bonferroni校正p<；0.05)。最好的MRS预测因子是通过树形分类程序选择的，并导致所有受试者89%的准确率，而患者组之间的SARA评分有相当大的重叠。因此，这项研究证实了SCA和MSA-C相对于对照组的多种神经化学改变，以及这些神经化学水平区分共济失调类型的可能性。有更多患者和其他共济失调患者的研究有必要进一步研究高场磁共振波谱作为共济失调生物标志物所测量的神经化学水平的临床应用。
Hereditary and sporadic neurodegenerative ataxias are movement disorders that affect the cerebellum. Robust and objective biomarkers are critical for treatment trials of ataxias. In addition, such biomarkers may help discriminate between ataxia subtypes because these diseases display substantial overlap in clinical presentation and conventional MRI. Profiles of 10–13 neurochemical concentrations obtained in vivo by high field proton magnetic resonance spectroscopy (1H MRS) can potentially provide ataxia-type specific biomarkers. We compared cerebellar and brainstem neurochemical profiles measured at 4 T from 26 patients with spinocerebellar ataxias (SCA1, N=9; SCA2, N=7; SCA6, N=5) or cerebellar multiple system atrophy (MSA-C, N=5) and 15 age-matched healthy controls. The Scale for the Assessment and Rating of Ataxia (SARA) was used to assess disease severity. The patterns of neurochemical alterations relative to controls differed between ataxia types. Myo-inositol levels in the vermis, myo-inositol, total N-acetylaspartate, total creatine, glutamate, glutamine in the cerebellar hemispheres and myo-inositol, total N-acetylaspartate, glutamate in the pons were significantly different between patient groups (Bonferroni corrected p<0.05). The best MRS predictors were selected by a tree classification procedure and lead to 89% accurate classification of all subjects while the SARA scores overlapped considerably between patient groups. Therefore, this study demonstrated multiple neurochemical alterations in SCAs and MSA-C relative to controls and the potential for these neurochemical levels to differentiate ataxia types. Studies with higher numbers of patients and other ataxias are warranted to further investigate the clinical utility of neurochemical levels as measured by high-field MRS as ataxia biomarkers.
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影响因子：
14.5
作者：
Guerrini, L;Lolli, F;Mascalchi, M
通讯作者：
Mascalchi, M
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3.5
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