Author:
Verde Federico,Steinacker Petra,Weishaupt Jochen H,Kassubek Jan,Oeckl Patrick,Halbgebauer Steffen,Tumani Hayrettin,von Arnim Christine A F,Dorst Johannes,Feneberg Emily,Mayer Benjamin,Müller Hans-Peter,Gorges Martin,Rosenbohm Angela,Volk Alexander E,Silani Vincenzo,Ludolph Albert C,Otto Markus
Abstract
ObjectiveTo determine the diagnostic and prognostic performance of serum neurofilament light chain (NFL) in amyotrophic lateral sclerosis (ALS).MethodsThis single-centre, prospective, longitudinal study included the following patients: 124 patients with ALS; 50 patients without neurodegenerative diseases; 44 patients with conditions included in the differential diagnosis of ALS (disease controls); 65 patients with other neurodegenerative diseases (20 with frontotemporal dementia, 20 with Alzheimer’s disease, 19 with Parkinson’s disease, 6 with Creutzfeldt-Jakob disease (CJD)). Serum NFL levels were measured using the ultrasensitive single molecule array (Simoa) technology.ResultsSerum NFL levels were higher in ALS in comparison to all other categories except for CJD. A cut-off level of 62 pg/mL discriminated between ALS and all other conditions with 85.5% sensitivity (95% CI 78% to 91.2%) and 81.8% specificity (95% CI 74.9% to 87.4%). Among patients with ALS, serum NFL correlated positively with disease progression rate (rs=0.336, 95% CI 0.14 to 0.506, p=0.0008), and higher levels were associated with shorter survival (p=0.0054). Serum NFL did not differ among patients in different ALS pathological stages as evaluated by diffusion-tensor imaging, and in single patients NFL levels were stable over time.ConclusionsSerum NFL is increased in ALS in comparison to other conditions and can serve as diagnostic and prognostic biomarker. We established a cut-off level for the diagnosis of ALS.
Funder
Foundation of the state of Baden-Wuerttemberg
Virtual Helmholz Institute “RNA Dysmetabolism in FTD and ALS”
Amyotrophic Lateral Sclerosis Association
Boehringer Ingelheim Ulm University BioCenter
German Federal Ministry of Education and Research
EU Joint Programme - Neurodegenerative Diseases network PreFrontAls
Deutsche Forschungsgemeinschaft
Thierry Latran Foundation
Subject
Psychiatry and Mental health,Neurology (clinical),Surgery
Cited by
170 articles.
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