ArticleNeurology2025
Clinical, Electrophysiologic, and Pathologic Features of Anti-Contactin-Associated Protein 1 Autoimmune Nodopathy.
Article in Neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers, 1 of them a synthesis that pooled it.
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Who cites it
3 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Efficacy and safety of anti-CD20 monoclonal antibody therapy for autoimmune nodopathies: a systematic review and meta-analysis.Frontiers in neurologyPooled it
- Refining Electrophysiological Criteria in CIDP and MMN: Toward a Unified Definition of Motor Conduction Block and Proximal Temporal Dispersion.European journal of neurology · 2026Article
- Guillain-Barré Syndrome: Progress in Diagnosis, Biomarkers, Neuroimaging and Management.ImmunoTargets and therapy · 2026Review
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Authors and funding
40 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesThe significance of anti-contactin-associated protein 1 (Caspr1) antibodies in autoimmune nodopathies (ANs) has not been fully established. The aim of this study was to elucidate the clinical profiles of Caspr1 AN.
methodsConsecutive serum samples were included from patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) nationwide who were referred to our laboratory for antibody testing with questionnaires as part of routine clinical practice and who fulfilled definite European Federation of Neurological Societies and Peripheral Nerve Society electrodiagnostic criteria. Anti-Caspr1 immunoglobulin (Ig) G was screened using in-house ELISA and confirmed using immunohistochemistry and Western blotting. Clinical data, electrophysiologic findings, and treatment responses were retrospectively collected. The pathologic features of sural nerves were also examined. Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores, antibody titers, and serum neurofilament light chain (NfL) levels were analyzed at baseline and follow-up.
resultsOf 559 screened serum samples from patients with CIDP (median age at onset 54 years, 35% female), 19 cases with anti-Caspr1 IgG were identified; the main subclass was IgG4 (17 cases). Patients with IgG4 Caspr1 AN cases exhibited an older age at disease onset (median 67 years); male predominance (82%); high proportions of limb weakness (100%), gait disturbance (100%), tremor (65%), and sensory ataxia (82%); and very high CSF protein levels (249 mg/dL). Nerve conduction studies showed prolonged F-wave and distal motor latencies and reduced sensorimotor conduction velocities in all nerves tested. Light microscopy findings of sural nerve biopsy specimens from 4 patients with Caspr1 AN indicated a loss of myelinated fiber density and myelin ovoids without macrophage-mediated demyelination or onion bulbs. Electron microscopic evaluation demonstrated axo-glial detachment. Patients with IgG4 Caspr1 AN showed a poor response to IVIg (31%) and needed combined immunotherapies. Even after correcting for age, serum NfL levels were higher in patients with IgG4 Caspr1 AN than in healthy controls ( DISCUSSION: IgG4 Caspr1 AN presents with a similar clinical phenotype to other nodopathies (e.g., neurofascin 155 and contactin 1), but with an older age at onset. Changes in antibody titers may be a potential biomarker for monitoring disease activity.
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