ArticleAnnals of neurology2018
Mechanisms of Caspr2 antibodies in autoimmune encephalitis and neuromyotonia.
Article in Annals of neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 63 papers, 1 of them a synthesis that pooled it.
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Who cites it
63 citing papers in PubMed, 1 synthesis or guideline pooled it, 113 citations in OpenAlex.
- Retrograde Amnesia in LGI1 and CASPR2 Limbic Encephalitis: Two Case Reports and a Systematic Literature Review.European journal of neurology · 2025Pooled it
- Antibody-Dependent Cellular Phagocytosis and Cytotoxicity in Patients With LGI1 and CASPR2 Encephalitis.Neurology(R) neuroimmunology & neuroinflammation · 2026Article
- Contrasting Outcomes in Two Cases of Anti-contactin-associated Protein-like 2 Autoimmune Encephalitis.Internal medicine (Tokyo, Japan) · 2026Article
- Blocking interaction of sclerostin loop3 with osteoblastic LRP4 counteracts bone loss without increasing arterial stiffness during mechanical unloading.Journal of orthopaedic translation · 2026Article
- Movement disorders with autoimmune neuromuscular origin: an overview of Isaacs' syndrome, stiff person syndrome, immune-mediated rippling muscle disease.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Synaptophysin autoantibodies mediate synaptic dysfunction in cerebellar ataxia.Cell reports. Medicine · 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
- Morvan Syndrome Masquerading as Anxiety Disorder: A Case Report Highlighting the Importance of Recognizing Organic Signs in Psychiatric Settings.International medical case reports journal · 2026Article
- Case Report: Dual response to efgartigimod in myasthenia gravis and comorbid autoimmune disorders: a case series.Frontiers in immunology · 2026Article
- CNTNAP2: isoform- and context-specific functions in neurological disorders and cancer.Frontiers in cellular neuroscience · 2026Review
- Multi-omics integration in autoimmune encephalitis: from novel biomarker discovery to precision therapeutic strategies.Frontiers in immunology · 2026Review
- Brain atrophy in autoimmune encephalitis: epidemiology, pathophysiology, clinical manifestations, treatment, and prognosis-an update.Journal of neurology · 2025Review
- Autoimmune encephalitis.Nature reviews. Disease primers · 2025Review
- Activated αβ T and reduced mucosa-associated invariant T cells in LGI1- and CASPR2-encephalitis.Brain : a journal of neurology · 2025Article
- Neuropathic Pain and Distinct CASPR2 Autoantibody IgG Subclasses Drive Neuronal Hyperexcitability.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Autoimmune encephalitis-associated epilepsy.Nature reviews. Neurology · 2025Review
- Neurodegeneration and the immune system: lessons from autoimmune encephalitis.Journal of neurology · 2025Review
- Permissive central tolerance plus defective peripheral checkpoints license pathogenic memory B cells in CASPR2-antibody encephalitis.Science advances · 2025Article
- Toward curing neurological autoimmune disorders: Biomarkers, immunological mechanisms, and therapeutic targets.Neuron · 2025Review
- The Discovery of Autoimmune Nodopathies and the Impact of IgG4 Antibodies in Autoimmune Neurology.Neurology(R) neuroimmunology & neuroinflammation · 2025Review
3 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
Abstract
objectiveTo determine the pathogenic mechanisms of autoantibodies to the cell adhesion molecule Caspr2 in acquired neuromyotonia and autoimmune encephalitis.
methodsCaspr2-positive samples were confirmed using a cell-based assay, and their IgG subtypes were determined by enzyme-linked immunosorbent assay and cell-based assay. A solid phase binding assay quantified the binding of Caspr2 to contactin-2 in the presence of Caspr2 autoantibodies. Living cultures of primary rat hippocampal neurons were incubated with Caspr2-positive or control sera, and the distribution of Caspr2-positive immunofluorescent puncta and total surface Caspr2 was quantified. HEK cells transfected to express Caspr2 were incubated with Caspr2-positive or control samples, and cell-surface biotinylation and Western blot were used to assess total, internalized, and surface levels of Caspr2.
resultsWe confirmed 6 samples with strong Caspr2 reactivity. IgG4 Caspr2 antibodies were present in all 6 cases. Caspr2 interacted with another cell adhesion molecule, contactin-2, with nanomolar affinity in the solid phase assay, and Caspr2 autoantibodies inhibited this interaction. Caspr2 autoantibodies did not affect the surface expression of Caspr2 in rat primary hippocampal neurons or transfected HEK cells.
interpretationCaspr2 autoantibodies inhibit the interaction of Caspr2 with contactin-2 but do not cause internalization of Caspr2. Functional blocking of cell adhesion molecule interactions represents a potential mechanism with therapeutic implications for IgG4 autoantibodies to cell adhesion molecules in neurological diseases. Ann Neurol 2018;83:40-51.
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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.