Serum neurofilament light chain predicts long-term prognosis in Guillain-Barré syndrome patients

Martín-Aguilar, Lorena; Camps‐Renom, Pol; Lleixà, Cinta; Pascual-Goñi, Elba; Díaz‐Manera, Jordi; Rojas‐García, Ricardo; Luna, Noemí de; Gallardo, Eduard; eacutes-Vicente, E. Cort; Muñoz, Laia; Alcolea, Daniel; Lleó, Alberto; Casasnovas, Carlos; Homedes, Christian; Gutiérrez‐Gutiérrez, Gerardo; Jimeno-Montero, María Concepción; Berciano, José; Sedano-Tous, M.J.; García-Sobrino, Tania; Pardo-Fernández, Julio; Márquez-Infante, Celedonio; Rojas-Marcos, Íñigo; Jerico-Pacual, I.; Martínez‐Hernández, Eugenia; Tassa, Germán Morís de la; Domínguez‐González, Cristina; Illa, Isabel; Querol, Luís

doi:10.1101/2020.03.24.20042200

Cited by 15 publications

(23 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 18 In our study, we found a strong correlation between baseline sNfL levels and initial I-RODS and maximum I-RODS achieved, but not with final I-RODS, suggesting that the final outcomes are not completely determined by initial severity because effective therapies change the course of the disease and most patients improve significantly, regardless of the treatment used. These data differ from those found in GBS, 24 a monophasic disorder in which initial events determine long-term outcomes, but nonetheless suggest that sNfL may be useful to monitor disease because it seems to happen in other peripheral neuropathies. 43 - 45 …”

Section: Discussioncontrasting

confidence: 92%

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

Martín-Aguilar

Lleixà²,

Pascual-Goñi³

et al. 2022

Neurol Neuroimmunol Neuroinflamm

Self Cite

View full text Add to dashboard Cite

Background and ObjectivesTo study the clinical and laboratory features of antineurofascin-155 (NF155)–positive autoimmune nodopathy (AN).MethodsPatients with anti-NF155 antibodies detected on routine immunologic testing were included. Clinical characteristics, treatment response, and functional scales (modified Rankin Scale [mRS] and Inflammatory Rasch-built Overall Disability Scale [I-RODS]) were retrospectively collected at baseline and at the follow-up. Autoantibody and neurofilament light (NfL) chain levels were analyzed at baseline and at the follow-up.ResultsForty NF155+ patients with AN were included. Mean age at onset was 42.4 years. Patients presented with a progressive (75%), sensory motor (87.5%), and symmetric distal-predominant weakness in upper (97.2%) and lower extremities (94.5%), with tremor and ataxia (75%). Patients received a median of 3 (2–4) different treatments in 46 months of median follow-up. Response to IV immunoglobulin (86.8%) or steroids (72.2%) was poor in most patients, whereas 77.3% responded to rituximab. HLA-DRB1*15 was detected in 91.3% of patients. IgG4 anti-NF155 antibodies were predominant in all patients; anti-NF155 titers correlated with mRS within the same patient (r = 0.41, p = 0.004). Serum NfL (sNfL) levels were higher in anti-NF155+ AN than in healthy controls (36.47 vs 7.56 pg/mL, p < 0.001) and correlated with anti-NF155 titers (r = 0.43, p = 0.001), with I-RODS at baseline (r = −0.88, p < 0.001) and with maximum I-RODS achieved (r = −0.58, p = 0.01). Anti-NF155 titers and sNfL levels decreased in all rituximab-treated patients.DiscussionAnti-NF155 AN presents a distinct clinical profile and good response to rituximab. Autoantibody titers and sNfL are useful to monitor disease status in these patients. The use of untagged-NF155 plasmids minimizes the detection of false anti-NF155+ cases.Classification of EvidenceThis study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.

show abstract

Section: Discussioncontrasting

confidence: 92%

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

Martín-Aguilar

Lleixà²,

Pascual-Goñi³

et al. 2022

Neurol Neuroimmunol Neuroinflamm

Self Cite

View full text Add to dashboard Cite

show abstract

“…Serum and CSF samples from patients with NMDARe and serum from patients of the other disease groups or HC were divided into aliquots and stored at −80°C until needed. Measurement of serum and CSF NfL levels was performed as reported 29 with the single-molecule array Nf-light kit in SR-X immunoassay analyzer (Quanterix Corp, Boston, MA), which runs ultrasensitive paramagnetic bead-based ELISAs. Samples were analyzed in duplicates by experienced personnel blinded to clinical diagnoses, following the manufacturer's instructions and standard procedures in Hospital de la Santa Creu i Sant Pau, Barcelona.…”

Section: Methodsmentioning

confidence: 99%

Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis

et al. 2022

Self Cite

View full text Add to dashboard Cite

Introduction:An important challenge in diagnosing anti-NMDAR encephalitis (NMDARe) is differentiating it from a first episode of psychosis (FEP) caused by a psychiatric disease (pFEP). CSF antibody testing distinguishes these diseases, but spinal taps are difficult to obtain in psychiatric facilities. A separate problem is the lack of biomarkers of NMDARe severity and outcome. Here we assessed the performance of neurofilament light chain (NfL) testing in these settings.Methods:In this observational study, NfL were determined with Single molecule array (SiMoA) in patients with NMDARe, pFEP, herpes simplex encephalitis (HSE), and healthy subjects (HC), the latter two groups used as controls. Receiver operating characteristic (ROC) analyses were performed to assess the prediction accuracy of serum NfL (sNfL) levels for NMDARe and pFEP, and to obtain clinically useful cutoffs.Results:118 patients with NMDARe (33 with isolated psychosis at presentation), 45 pFEP, 36 HSE, and 36 HC were studied. NMDARe patients with seizures/status epilepticus, ICU admission, CSF pleocytosis (>20 WBC/µL), and without early immunotherapy were more likely to have higher sNfL than NMDARe without these features. NfL levels at diagnosis of NMDARe did not correlate with outcome at 1 year follow-up assessed with the modified Ranking Scale (mRS). NMDARe patients had significantly higher NfL than pFEP and HC, and lower than HSE patients. ROC analysis of sNfL between NMDARe with isolated psychosis and pFEP provided an AUC of 0.93 (95% CI 0.87-0.99) and a sNfL cutoff ≥15 pg/mL to distinguish these disorders (sensitivity 85%, specificity 96%, positive likelihood ratio 19.3). Forty-three/45 (96%) pFEP had sNfL<15pg/mL whereas only 5/33 (15%) NMDARe with isolated psychosis were below this cutoff (risk estimation NMDARe vs pFEP: odds ratio 120.4 [95% CI 21.8-664], p<0.001). None of the HSE and 35/36 (97%) HC had sNfL<15pg/mL.Discussion:NfL measured at diagnosis of NMDARe associated with features of disease severity but not with long-term outcome. Young patients with FEP and sNfL≥15pg/mL had 120 times higher chance of having NMDARe than pFEP. This cutoff correctly classified 96% of pFEP and 85% of NMDARe with isolated psychosis. Patients with FEP of unclear etiology and sNfL≥15pg/mL should undergo CSF NMDAR-antibody testing.

show abstract

“…Taken together, sNfL could be a biomarker of disease activity and disability but might not influence the course of an NMOSD attack. It is noteworthy sNfL showed predictive value for long-term clinical outcomes in multiple sclerosis and Guillain-Barré syndrome ( 39 , 40 ).…”

Section: Discussionmentioning

confidence: 99%

Therapeutic Response and Possible Biomarkers in Acute Attacks of Neuromyelitis Optica Spectrum Disorders: A Prospective Observational Study

Wang

Chu

et al. 2021

Front. Immunol.

View full text Add to dashboard Cite

ObjectiveTo explore the outcomes of NMOSD attacks and investigate serum biomarkers for prognosis and severity.MethodPatients with NMOSD attacks were prospectively and observationally enrolled from January 2019 to December 2020 at four hospitals in Guangzhou, southern China. Data were collected at attack, discharge and 1/3/6 months after acute treatment. Serum cytokine/chemokine and neurofilament light chain (NfL) levels were examined at the onset stage.ResultsOne hundred patients with NMOSD attacks were included. The treatment comprised intravenous methylprednisolone pulse therapy alone (IVMP, 71%), IVMP combined with apheresis (8%), IVMP combined with intravenous immunoglobulin (18%) and other therapies (3%). EDSS scores decreased significantly from a medium of 4 (interquartile range 3.0–5.5) at attack to 3.5 (3.0–4.5) at discharge, 3.5 (2.0–4.0) at the 1-month visit and 3.0 (2.0–4.0) at the 3-month visit (p<0.01 in all comparisons). The remission rate was 38.0% at discharge and 63.3% at the 1-month visit. Notably, relapse occurred in 12.2% of 74 patients by the 6-month follow-up. Higher levels of T helper cell 2 (Th2)-related cytokines, including interleukin (IL)-4, IL-10, IL-13, and IL-1 receptor antagonist, predicted remission at the 1-month visit (OR=9.33, p=0.04). Serum NfL levels correlated positively with onset EDSS scores in acute-phase NMOSD (p<0.001, R2 = 0.487).ConclusionsOutcomes of NMOSD attacks were generally moderate. A high level of serum Th2-related cytokines predicted remission at the 1-month visit, and serum NfL may serve as a biomarker of disease severity at attack.Clinical Trial Registrationhttps://clinicaltrials.gov/ct2/show/NCT04101058, identifier NCT04101058.

show abstract

Serum neurofilament light chain predicts long-term prognosis in Guillain-Barré syndrome patients

Cited by 15 publications

References 28 publications

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis

Therapeutic Response and Possible Biomarkers in Acute Attacks of Neuromyelitis Optica Spectrum Disorders: A Prospective Observational Study

Contact Info

Product

Resources

About