TDP-43 as a potential biomarker for amyotrophic lateral sclerosis: a systematic review and meta-analysis

BMC Neurology

卷:18

Vivek Majumder¹  Jenna M. Gregory¹  Suvankar Pal¹  Marcelo A. Barria²  Alison Green² 

[1] Centre for Clinical Brain Sciences, University of Edinburgh;

[2] National CJD Research and Surveillance Unit, Bryan Matthews Building, Western General Hospital;

关键词: TDP-43;    Amyotrophic lateral sclerosis;    Biomarker;    Systematic review;    Meta-analysis;   

DOI  :  10.1186/s12883-018-1091-7

来源: DOAJ

【 摘 要 】

Abstract Background Frontotemporal dementia (FTD) and Amyotrophic Lateral Sclerosis (ALS) are incurable, progressive and fatal neurodegenerative diseases with patients variably affected clinically by motor, behavior, and cognitive deficits. The accumulation of an RNA-binding protein, TDP-43, is the most significant pathological finding in approximately 95% of ALS cases and 50% of FTD cases, and discovery of this common pathological signature, together with an increasing understanding of the shared genetic basis of these disorders, has led to FTD and ALS being considered as part of a single disease continuum. Given the widespread aggregation and accumulation of TDP-43 in FTD-ALS spectrum disorder, TDP-43 may have potential as a biomarker in these diseases. Methods We therefore conducted a systematic review and meta-analysis to evaluate the diagnostic utility of TDP-43 detected in the cerebrospinal fluid (CSF) of patients with FTD-ALS spectrum disorder. Results From seven studies, our results demonstrate that patients with ALS have a statistically significantly higher level of TDP-43 in CSF (effect size 0.64, 95% CI: 0.1–1.19, p = 0.02). Conclusions These data suggest promise for the use of CSF TDP-43 as a biomarker for ALS.

【 授权许可】

Unknown