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Background: International consensus on IgM ± anti‐MAG ± PNP (IgM PNP) is lacking. Despite increasing interest in clinical trials, validated disease‐specific measures are needed to adequately capture limitations and changes over time. The IMAGiNe (IgM ± anti‐myelin associated glycoprotein [MAG] peripheral neuropathy) study surges as an international collaboration to create a standardized registry of patients with IgM ± anti‐MAG PNP. The consortium, which currently consists of 11 institutions from 7 countries, presents here the IMAGiNe study design and protocol. Aims: Functional outcome measures will be constructed at the level of impairment, as well as activity and participation. We aim to describe the natural history of the cohort, the role of anti‐MAG antibodies, the presence of clinical subtypes, and potential biomarkers. Methods: The IMAGiNe study is a prospective, observational cohort study with a 3‐year follow‐up. At each assessment, researchers collect clinical data and subjects complete a list of preselected outcome measures. Among these, the "Pre‐Rasch‐built Overall Disability Scale (Pre‐RODS)" questionnaire will be submitted to Rasch analysis to assess classic and modern clinimetric requirements. Results: The final measures will include the IgM‐PNP‐specific RODS and Ataxia Rating Scale (IgM‐PNP‐ARS). Descriptions of the disease course, clinical heterogeneity, treatment regimes, variations in laboratory values, and antibody titers will help reach consensus on diagnosis and follow‐up strategies. Conclusion: The constructed interval scales will be cross‐culturally valid and suitable for use in future clinical trials and daily practice. The ultimate goals are to improve functional individualized assessment, reach international consensus, and lay the foundations for successful designs in future studies. [ABSTRACT FROM AUTHOR]

Introduction: Polyneuropathy with immunoglobulin M monoclonal gammopathy (IgM-PNP) is associated with the presence of IgM antibodies against nerve constituents such as myelin associated glycoprotein (MAG) and gangliosides.Methods: To test whether B-cell-stimulating cytokines are increased in IgM-PNP, we measured serum concentrations of 11 cytokines in 81 patients with IgM-PNP and 113 controls.Results: Median interleukin (IL)-6 concentrations were higher in patients with IgM-PNP, and median IL-10 concentrations were higher in the subgroup with anti-MAG IgM antibodies. These serum concentrations were not increased in 110 patients with multifocal motor neuropathy.Discussion: Median IL-6 and IL-10 serum concentrations differ between patients with anti-MAG neuropathy and other patients with IgM-PNP compared with healthy and neuropathy controls. These differences may indicate differences in immune-mediated disease mechanisms. Muscle Nerve 59:694-698, 2019. [ABSTRACT FROM AUTHOR]

OBJECTIVE--This study aims to investigate the association between chronic idiopathic axonal polyneuropathy (CIAP) and the metabolic syndrome or its individual components. RESEARCH DESIGN AND METHODS--A total of 249 patients with CIAP and 709 controls underwent fasting laboratory studies, and blood pressure and waist circumference were measured. The metabolic syndrome was diagnosed if three or more of the following Adult Treatment Panel III criteria were present: impaired fasting glucose, hypertension, abdominal obesity, reduced HDL cholesterol, and hypertriglyceridemia. Subgroup analysis was performed for patients with a painful predominantly sensory CIAP, because this phenotype is most similar to diabetic polyneuropathy. Statistical analysis was performed with adjustment for age and gender. RESULTS--Fifty-five percent of all patients fulfilled themetabolic syndrome criteria compared with 34% of controls (odds ratio 2.2 [95% CI 1.7-3.0]). Multivariate analysis shows hypertension (2.9 [1.7-4.9]) and abdominal obesity (3.3 [2.4-4.6]) to be significantly more prevalent in patients than in controls. Of the patients classified as having a painful predominantly sensory CIAP, 62% fulfilled the metabolic syndrome criteria (3.1 [2.0-4.8]). In this subgroup, hypertension and abdominal obesity also were significantly more prevalent compared with controls. CONCLUSIONS--Abdominal obesity and hypertension seem to be the most consistent contributing components of the metabolic syndrome in patients with CIAP. Evaluation and appropriate treatment of these risk factors in patients with CIAP would be advocated. [ABSTRACT FROM AUTHOR]

Abstract: IgM antibodies against gangliosides and their complexes were studied in sera from 54 patients with polyneuropathy and IgM monoclonal gammopathy (IgM-PNP) without anti-MAG antibodies. Anti-ganglioside antibodies were found in 19 (35%) patients. Five (9%) patients had antibodies against ganglioside complexes. IgM antibodies against gangliosides activated complement in vitro. Light chain usage was restricted to kappa or lambda in most, but not all patients. In conclusion, anti-ganglioside antibodies in IgM-PNP are common, display pathogenic properties and do not always arise from a monoclonal B cell proliferation. [Copyright &y& Elsevier]