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Background and Purpose: The purpose was to explore the prognostic utility of neurofilament light chain (NfL) in patients with immune-mediated polyradiculoneuropathies (IMPs)., Methods: This retrospective monocentric study analysed serum and cerebrospinal fluid samples from patients diagnosed with IMP collected prior to treatment initiation. NfL concentrations were correlated with clinical outcomes, including F score and hospitalization duration., Results: Amongst 115 IMP patients tested, baseline cerebrospinal fluid and serum NfL (sNfL) concentrations were higher in acute inflammatory axonal polyradiculoneuropathy (AIAP) than other IMP variants. In the AIAP cohort, a positive correlation was observed between baseline sNfL concentrations, F score and hospitalization duration. Multivariate linear regression analysis further supported the predictive relationship between elevated baseline sNfL concentrations and clinical outcomes. Using receiver operating characteristic analysis, a cut-off value for sNfL of 351 pg/mL was found to predict an F score >3 in AIAP with a sensitivity of 40% and specificity of 81.8%. AIAP patients with sNfL concentrations above this threshold required longer hospitalization (extended by 15 days)., Discussion: Our findings highlight the potential of baseline sNfL as an effective marker for distinguishing between IMP variants and predicting the prognosis of AIAP. Further validation may facilitate translation of sNfL into clinical practice, potentially identifying high-risk patients for tailored treatment approaches., (© 2024 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background and Purpose: With the new highly active drugs available for people with multiple sclerosis (pwMS), vaccination becomes an essential part of the risk management strategy. We aimed to develop a European evidence-based consensus for the vaccination strategy of pwMS who are candidates for disease-modifying therapies (DMTs)., Methods: This work was conducted by a multidisciplinary working group using formal consensus methodology. Clinical questions (defined as population, interventions and outcomes) considered all authorized DMTs and vaccines. A systematic literature search was conducted and quality of evidence was defined according to the Oxford Centre for Evidence-Based Medicine Levels of Evidence. The recommendations were formulated based on the quality of evidence and the risk-benefit balance., Results: Seven questions, encompassing vaccine safety, vaccine effectiveness, global vaccination strategy and vaccination in subpopulations (pediatric, pregnant women, elderly and international travelers) were considered. A narrative description of the evidence considering published studies, guidelines and position statements is presented. A total of 53 recommendations were agreed by the working group after three rounds of consensus., Conclusion: This first European consensus on vaccination in pwMS proposes the best vaccination strategy according to current evidence and expert knowledge, with the goal of homogenizing the immunization practices in pwMS., (© 2023 The Authors. Co-published by European Academy of Neurology and European Committee of Treatment of Research in Multiple Sclerosis.)

Background and Purpose: Thinning of the retinal combined ganglion cell and inner plexiform layer (GCIP) as measured by optical coherence tomography (OCT) is a common finding in patients with multiple sclerosis. This study aimed to investigate whether a single retinal OCT analysis allows prediction of future disease activity after a first demyelinating event., Methods: This observational cohort study included 201 patients with recently diagnosed clinically isolated syndrome or relapsing-remitting multiple sclerosis from two German tertiary referral centers. Individuals underwent neurological examination, magnetic resonance imaging, and OCT at baseline and at yearly follow-up visits., Results: Patients were included at a median disease duration of 2.0 months. During a median follow-up of 59 (interquartile range = 43-71) months, 82% of patients had ongoing disease activity as demonstrated by failing the no evidence of disease activity 3 (NEDA-3) criteria, and 19% presented with confirmed disability worsening. A GCIP threshold of ≤77 μm at baseline identified patients with a high risk for NEDA-3 failure (hazard ratio [HR] = 1.7, 95% confidence interval [CI] = 1.1-2.8, p = 0.04), and GCIP measures of ≤69 μm predicted disability worsening (HR = 2.2, 95% CI = 1.2-4.3, p = 0.01). Higher rates of annualized GCIP loss increased the risk for disability worsening (HR = 2.5 per 1 μm/year increase of GCIP loss, p = 0.03)., Conclusions: Ganglion cell thickness as measured by OCT after the initial manifestation of multiple sclerosis may allow early risk stratification as to future disease activity and progression., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background and Purpose: Peripapillary hyper-reflective ovoid masslike structures (PHOMS) are a novel finding during retinal optical coherence tomography in patients with multiple sclerosis (MS). To date, there are no data on the occurrence of PHOMS in early MS. The aim of this study was to investigate the frequency of PHOMS in patients with first diagnosed early relapsing-remitting MS (RRMS) and to search for associations of PHOMS with disease patterns in different MS subtypes., Methods: This was a cross-sectional analysis in two different cohorts: cohort 1, consisting of early RRMS patients (n = 349); cohort 2, consisting of patients with primary progressive MS (PPMS) (n = 66) and RRMS (n = 65)., Results: Peripapillary hyper-reflective ovoid masslike structures were detected in 18.3% of patients with early RRMS. The occurrence of PHOMS was not associated with age, disease duration and disability. Investigating clinical patterns and the occurrence of PHOMS (cohort 2), an association of PHOMS with higher Expanded Disability Status Scale measures (PHOMS 4.9, 3.7-6.1; no PHOMS 3.5, 3.0-5.3; p = 0.03) and longer disease durations (PHOMS 6.5 years, 1.9-11.0; no PHOMS 1.0 years, 0.0-4.0, p = 0.0007) was found in patients with PPMS but not RRMS. After p value adjustment, the disease duration appeared to be more relevant (β = 0.16, p = 0.06)., Conclusion: Peripapillary hyper-reflective ovoid masslike structures were found in 18% of patients with early MS. The presence of PHOMS might be associated with disease progression only in PPMS but not RRMS, suggesting that PHOMS might be embedded in neurodegenerative processes., (© 2021 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background and Purpose: Multiple sclerosis (MS) is a complex disease of the central nervous system. As new drugs are becoming available, knowledge on diagnosis and treatment must continuously evolve. There is therefore a need for a reference tool compiling current data on benefit and safety, to aid professionals in treatment decisions and use of resources across Europe. The European Committee of Treatment and Research in Multiple Sclerosis (ECTRIMS) and the European Academy of Neurology (EAN) have joined forces to meet this need. The objective was to develop an evidence-based clinical practice guideline for the pharmacological treatment of people with MS to guide healthcare professionals in the decision-making process., Methods: This guideline has been developed using the GRADE methodology and following the recently updated EAN recommendations for guideline development. Clinical questions were formulated in PICO format (patient, intervention, comparator, outcome) and outcomes were prioritized according to their relevance to clinical practice. An exhaustive literature search up to December 2016 was performed for each question and the evidence is presented narratively and, when possible, combined in a meta-analysis using a random-effects model. The quality of evidence for each outcome was rated into four categories - very high, high, low and very low - according to the risk of bias. GRADE evidence profiles were created using GRADEprofiler (GRADEpro) software (Version 3.6). The recommendations with assigned strength (strong, weak) were formulated based on the quality of evidence and the risk-benefit balance. Consensus between the panellists was reached by use of the modified nominal group technique., Results: A total of 10 questions have been agreed, encompassing treatment efficacy, response criteria, strategies to address suboptimal response and safety concerns and treatment strategies in MS and pregnancy. The guideline takes into account all disease-modifying drugs approved by the European Medicine Agency at the time of publication. A total of 20 recommendations were agreed by the guideline working group members after three rounds of consensus., (© 2018 European Academy of Neurology and European Committee of Treatment of Research in Multiple Sclerosis.)