ArticleNeurology2014
Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg.
Article in Neurology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 154 papers, 7 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
154 citing papers in PubMed, 7 syntheses or guidelines pooled it, 359 citations in OpenAlex.
- Characterization of the neuromuscular manifestations in patients with IgG4-related disease: a systematic review.Immunologic research · 2025Pooled it
- Brazilian Academy of Neurology recommendations for diagnosis, management, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Arquivos de neuro-psiquiatria · 2025Guideline
- A Systematic Review and Meta-Analysis of Autoantibodies for Diagnosis and Prognosis in Patients With Chronic Inflammatory Demyelinating Polyradiculoneuropathy.Frontiers in neuroscience · 2021Pooled it
- Association of neurofascin IgG4 and atypical chronic inflammatory demyelinating polyneuropathy: A systematic review and meta-analysis.Brain and behavior · 2018Pooled it
- Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.The Cochrane database of systematic reviews · 2017Pooled it
- Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.The Cochrane database of systematic reviews · 2015Pooled it
- Efficacy and safety of anti-CD20 monoclonal antibody therapy for autoimmune nodopathies: a systematic review and meta-analysis.Frontiers in neurologyPooled it
- The prevalence of anti-neurofascin-155 antibodies in patients with neuromyelitis optica spectrum disorders.Clinical and experimental immunology · 2021Trial
- Randomized Placebo-Controlled Trial of Intravenous Immunoglobulin in Autoimmune LGI1/CASPR2 Epilepsy.Annals of neurology · 2020Trial
- Comparative Analytical Performance of Autoimmune Nodopathy Diagnostic Assays.Annals of laboratory medicine · 2026Article
- Neurofascin Antibodies in a Large Cohort of Inflammatory Neuropathies from India.Annals of Indian Academy of Neurology · 2026Article
- Clinical features and pathogenesis of Sjogrens disease related peripheral neuropathy and their relevance to clinical trials.Communications medicine · 2026Review
- A Next-Generation ELISA for the Detection of Anti-(Para)Nodal Antibodies in Autoimmune Nodopathy and COVID-19 Vaccinated Individuals.Journal of the peripheral nervous system : JPNS · 2026Article
- Clinical and laboratory features of juvenile-onset anti-NF155 autoimmune nodopathy.Journal of neurology, neurosurgery, and psychiatry · 2026Article
- Clinical and immunological features of auto-immune nodopathies.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026Article
- Clinical utility of human leukocyte antigen genotyping and immunoglobulin G4 autoantibody testing in autoimmune neurological diseases: A focused minireview.World journal of biological chemistry · 2026Review
- Case report: Unilateral optic atrophy and intracranial hypertension in anti-NF155 nodopathy: a case managed with sequential efgartigimod and rituximab.Frontiers in immunology · 2026Article
- Advances in the treatment of autoimmune nodopathy: based on treatment strategies of CIDP.Frontiers in immunology · 2026Review
- Evaluating the short-term and long-term therapeutic effects of immunoadsorption compared with plasma exchange in chronic inflammatory demyelinating polyneuropathy: a long-term, prospective, observational study.EClinicalMedicine · 2026Article
- Clinical, Electrophysiologic, and Pathologic Features of Anti-Contactin-Associated Protein 1 Autoimmune Nodopathy.Neurology · 2025Article
94 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
12 authors at 1 institution in 1 country.
Funding
Abstract
objectiveTo describe the frequency of antibodies against neurofascin in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and the associated clinical features.
methodsImmunocytochemistry was used to identify antibodies to neurofascin 155 (NF155) and 186. Serum reactivity with paranodes and brain tissue was tested with immunohistochemistry of teased-nerve fibers and rat brain. Antibody titers and immunoglobulin (Ig) G isotypes were determined using ELISA. Clinical information was obtained retrospectively.
resultsTwo of 53 patients, but none of 204 controls, had antibodies to NF155 (p = 0.041). The 2 patients with NF155 antibodies developed severe polyradiculoneuropathy with predominant distal weakness that was refractory to IVIg. Eight additional patients with IVIg-refractory CIDP were then identified from a national database; 2 of them with the same clinical features also had NF155 antibodies. Overall, 3 of the 4 patients with NF155 antibodies had a disabling and characteristic tremor (high amplitude, low frequency, postural, and intention). Patients' antibodies reacted with the paranodes in teased-nerve fibers and with the neuropil of rat cerebellum, brain, and brainstem. Anti-NF155 antibodies were predominantly of the IgG4 isotype in all patients.
conclusionPatients with CIDP positive for IgG4 NF155 antibodies constitute a specific subgroup with a severe phenotype, poor response to IVIg, and disabling tremor. Autoantibodies against paranodal structures associate with distinct clinical features in CIDP and their identification has diagnostic, prognostic, and therapeutic implications. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that autoantibodies to NF155 identify a CIDP subtype characterized by severe neuropathy, poor response to IVIg, and disabling tremor.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.