Department of Neurology, Erasmus Medical Center Rotterdam, The Netherlands; Department of Clinical Genetics, Leiden University Medical Center, The Netherlands.
Department of Neurology, Erasmus Medical Center Rotterdam, The Netherlands.
Parkinsonism Relat Disord. 2018 Nov;56:98-101. doi: 10.1016/j.parkreldis.2018.06.018. Epub 2018 Jun 12.
Neurofilament light chain (NfL) is a promising biomarker in neurodegenerative diseases. Elevated NfL levels in CSF and blood have been observed in a growing number of neurodegenerative disorders, including frontotemporal dementia and Alzheimer's disease. We studied serum NfL levels in patients with progressive supranuclear palsy (PSP) in relation to disease severity and survival.
Serum NfL levels were determined cross-sectionally in a retrospective cohort of 131 patients with PSP and 95 healthy controls. Detailed clinical examination was performed and disease severity was assessed by several rating scales.
We found that serum NfL levels in PSP were twice as high as those in controls, and that NfL levels correlated with worse functional, motor and cognitive functioning. During follow-up, 119 PSP patients had died, and higher NfL levels were associated with a shorter survival.
This study provides evidence that serum NfL is a relevant and promising biomarker in PSP for disease severity, and may be used as a prognostic tool to predict survival in clinical practice.
神经丝轻链（NfL）是神经退行性疾病中很有前途的生物标志物。越来越多的神经退行性疾病，包括额颞叶痴呆和阿尔茨海默病，都观察到脑脊液和血液中 NfL 水平升高。我们研究了进展性核上性麻痹（PSP）患者的血清 NfL 水平与疾病严重程度和存活的关系。
我们对 131 名 PSP 患者和 95 名健康对照者进行了回顾性队列研究，对血清 NfL 水平进行了横断面测定。进行了详细的临床检查，并使用几种评分量表评估了疾病严重程度。
我们发现 PSP 患者的血清 NfL 水平是对照组的两倍，NfL 水平与较差的功能、运动和认知功能相关。在随访期间，119 名 PSP 患者死亡，较高的 NfL 水平与较短的存活时间相关。
本研究提供了证据，表明血清 NfL 是 PSP 中一种相关且有前途的生物标志物，可用于评估疾病严重程度，并可能作为一种预后工具，用于预测临床实践中的存活情况。
