Cerebrospinal Fluid Biomarkers in Spinocerebellar Ataxia: A Pilot Study.
Cerebrospinal Fluid Biomarkers in Spinocerebellar Ataxia: A Pilot Study.
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DOI：
10.1155/2015/413098
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
Gomez CM
中科院分区：
文献类型：
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作者：
Brouillette AM;Öz G;Gomez CM
神经退行性疾病，包括脊髓小脑共济失调 (SCA)，将受益于可靠生物标志物的鉴定，这些生物标志物可以作为疾病亚型特异性和阶段特异性指标，用于治疗的开发和监测。我们分析了常染色体显性遗传性 SCA1、SCA2 和 SCA6 以及散发性疾病小脑型多系统萎缩症 (MSA-C) 患者的脑脊液 (CSF) 中 tau、α-突触核蛋白、DJ-1 和神经胶质纤维酸性蛋白 (GFAP) 的水平，这些蛋白质先前与神经退行性过程相关。我们使用共济失调评估和评级量表 (SARA) 估计疾病的严重程度。与对照组相比，疾病组中测量的大多数蛋白质呈较高趋势，但未达到统计学显着性。我们发现 SCA2 和 MSA-C 患者的 tau 水平显着高于对照组。我们发现，SCA1 中 SARA 得分越高，α-突触核蛋白水平越低；而 MSA-C 中 SARA 得分越高，tau 水平越高，尽管事后校正后这种最终相关性并未达到统计学显着性。需要进行更大样本量的其他研究来提高这些研究的功效并验证 CSF 生物标志物在 SCA 和 MSA-C 中的使用。
Neurodegenerative diseases, including the spinocerebellar ataxias (SCA), would benefit from the identification of reliable biomarkers that could serve as disease subtype-specific and stage-specific indicators for the development and monitoring of treatments. We analyzed the cerebrospinal fluid (CSF) level of tau, α-synuclein, DJ-1, and glial fibrillary acidic protein (GFAP), proteins previously associated with neurodegenerative processes, in patients with the autosomal dominant SCA1, SCA2, and SCA6, and the sporadic disease multiple system atrophy, cerebellar type (MSA-C), compared with age-matched controls. We estimated disease severity using the Scale for the Assessment and Rating of Ataxia (SARA). Most proteins measured trended higher in disease versus control group yet did not reach statistical significance. We found the levels of tau in both SCA2 and MSA-C patients were significantly higher than control. We found that α-synuclein levels were lower with higher SARA scores in SCA1 and tau levels were higher with greater SARA in MSA-C, although this final correlation did not reach statistical significance after post hoc correction. Additional studies with larger sample sizes are needed to improve the power of these studies and validate the use of CSF biomarkers in SCA and MSA-C.