Abstract：
Distinguishing behavioural variant frontotemporal dementia (bvFTD) from non-neurodegenerative \'non-progressor\' mimics of frontal lobe dysfunction, can be one of the most challenging clinical dilemmas. A biomarker of neuronal injury, neurofilament light chain (NfL), could reduce misdiagnosis and delay.
Cerebrospinal fluid (CSF) NfL, amyloid beta 1-42 (AB42), total and phosphorylated tau (T-tau, P-tau) levels were examined in patients with an initial diagnosis of bvFTD. Based on follow-up information, patients were categorised as Progressors or Non-Progressors: further subtyped into Non-Progressor Revised (non-neurological/neurodegenerative final diagnosis), and Non-Progressor Static (static deficits, not fully explained by non-neurological/neurodegenerative causes).
Forty-three patients were included: 20 Progressors, 23 Non-Progressors (15 Non-Progressor Revised, 8 Non-Progressor Static), and 20 controls. NfL concentrations were lower in Non-Progressors (Non-Progressors Mean, M = 554 pg/mL, 95%CI:[461, 675], Non-Progressor Revised M = 459 pg/mL, 95%CI:[385, 539], and Non-Progressor Static M = 730 pg/mL, 95%CI:[516, 940]), compared to Progressors (M = 2397 pg/mL, 95%CI:[1607, 3332]). NfL distinguished Progressors from Non-Progressors with the highest accuracy (area under the curve 0.92, 90%/87% sensitivity/specificity, 86%/91% positive/negative predictive value, 88% accuracy). Non-Progressor Static tended to have higher T-tau and P-tau levels compared to Non-Progressor Revised Diagnoses.
This study demonstrated strong diagnostic utility of CSF NfL to distinguish bvFTD from non-progressor variants, at baseline, with high accuracy, in a real-world clinical setting. This has important clinical implications, to improve outcomes for patients and clinicians facing this challenging clinical dilemma, healthcare services, and clinical trials. Further research is required to investigate heterogeneity within the non-progressor group and potential diagnostic algorithms, and prospective studies are underway assessing plasma NfL.
Cerebrospinal fluid (CSF) NfL, amyloid beta 1-42 (AB42), total and phosphorylated tau (T-tau, P-tau) levels were examined in patients with an initial diagnosis of bvFTD. Based on follow-up information, patients were categorised as Progressors or Non-Progressors: further subtyped into Non-Progressor Revised (non-neurological/neurodegenerative final diagnosis), and Non-Progressor Static (static deficits, not fully explained by non-neurological/neurodegenerative causes).
Forty-three patients were included: 20 Progressors, 23 Non-Progressors (15 Non-Progressor Revised, 8 Non-Progressor Static), and 20 controls. NfL concentrations were lower in Non-Progressors (Non-Progressors Mean, M = 554 pg/mL, 95%CI:[461, 675], Non-Progressor Revised M = 459 pg/mL, 95%CI:[385, 539], and Non-Progressor Static M = 730 pg/mL, 95%CI:[516, 940]), compared to Progressors (M = 2397 pg/mL, 95%CI:[1607, 3332]). NfL distinguished Progressors from Non-Progressors with the highest accuracy (area under the curve 0.92, 90%/87% sensitivity/specificity, 86%/91% positive/negative predictive value, 88% accuracy). Non-Progressor Static tended to have higher T-tau and P-tau levels compared to Non-Progressor Revised Diagnoses.
This study demonstrated strong diagnostic utility of CSF NfL to distinguish bvFTD from non-progressor variants, at baseline, with high accuracy, in a real-world clinical setting. This has important clinical implications, to improve outcomes for patients and clinicians facing this challenging clinical dilemma, healthcare services, and clinical trials. Further research is required to investigate heterogeneity within the non-progressor group and potential diagnostic algorithms, and prospective studies are underway assessing plasma NfL.
摘要：
区分行为变异性额颞叶痴呆（bvFTD）与非神经退行性非进展性额叶功能障碍的模拟，可能是最具挑战性的临床困境之一。神经元损伤的生物标志物，神经丝轻链（NfL），可以减少误诊和延误。
脑脊液(CSF)NfL,淀粉样β1-42（AB42），总和磷酸化tau(T-tau，在最初诊断为bvFTD的患者中检查P-tau）水平。根据后续信息，患者被分类为进展因子或非进展因子：进一步细分为非进展因子修订版（非神经系统/神经退行性最终诊断），和非进步静态(静态赤字，非神经系统/神经退行性原因不能完全解释)。
纳入43例患者：20名进展者，23个非前进项(15个非前进项修订，8非累进器静态)，20个控件非进展者的NfL浓度较低(非进展者平均值，M=554pg/mL，95CI:[461,675],非进展子修订后的M=459pg/mL，95CI:[385,539],和非渐进式静态M=730pg/mL，95CI:[516,940]),与进步因子相比(M=2397pg/mL，95CI：[1607，3332]）。NfL以最高的准确性区分进展者和非进展者（曲线下面积0.92，90%/87%的敏感性/特异性，86%/91%阳性/阴性预测值，88%的准确度)。与非前进子修订诊断相比，非前进子静态倾向于具有更高的T-tau和P-tau水平。
这项研究证明了CSFNfL在区分bvFTD与非进展变体方面的强大诊断效用，在基线，精度高，在现实世界的临床环境中。这具有重要的临床意义，为了改善面临这一具有挑战性的临床困境的患者和临床医生的预后，医疗保健服务,和临床试验。需要进一步的研究来调查非进展者组内的异质性和潜在的诊断算法，前瞻性研究正在评估血浆NfL。
脑脊液(CSF)NfL,
纳入43例患者：20名进展者，
这项研究证明了CSFNfL在区分bvFTD与非进展变体方面的强大诊断效用，
