Copine‐4 Paraneoplastic Autoimmune Encephalitis
We characterize copine-4 (CPNE4)-IgG and report clinical associations. Among 844 samples with unclassified neural IgGs identified by mouse-brain indirect immunofluorescence assay over 2 years, 5 samples (serum, 3; CSF, 2) from 3 patients produced a distinctive cerebrum-restricted synaptic pattern. Protein microarray disclosed CPNE4 "top hit" in those 3 patients; specificity was confirmed by 3 CPNE4-specific assays (confocal IFA, cell-based, and western blot). Median onset age was 67 years (range 60-68); all had encephalitis: a brainstem syndrome during immune checkpoint inhibitor therapy for lung cancer (2) and anti-NMDA-receptor encephalitis accompanied by neuroectoderm-lineage cancer (1). To date, CPNE4-IgG has paraneoplastic encephalitis significance.
Authors
- Michael Gilligan (ORCID: https://orcid.org/0000-0002-5656-3270)
- Αναστασία Ζεκερίδου (ORCID: https://orcid.org/0000-0002-1960-9065)
- Divyanshu Dubey (ORCID: https://orcid.org/0000-0001-6865-9045)
- Andrew B. McKeon (ORCID: https://orcid.org/0000-0001-6856-8143)
- Connie E. Lesnick (ORCID: https://orcid.org/0009-0008-2812-1214)
- Amir Adeli (ORCID: https://orcid.org/0000-0002-3355-3459)
- Diana Anissian
- Amy M.L. Quek (ORCID: https://orcid.org/0000-0002-0569-0472)
- John R. Mills (ORCID: https://orcid.org/0000-0003-1025-4760)
- Sean Joseph Pittock (ORCID: https://orcid.org/0000-0002-6140-5584)
- Clement Yong (ORCID: https://orcid.org/0000-0001-7060-3649)
- Gabrielle N. Conway
- Mimi S. Fang
Institutions
- Mayo Clinic (US)
- National University of Singapore (SG)
- The Ohio State University Wexner Medical Center (US)
- National University Hospital (SG)
- Marshall University (US)
Publication Details
- Journal
- Annals of Clinical and Translational Neurology
- Published
- 2026-10-04
- DOI
- https://doi.org/10.1002/acn3.70545
- Primary Topic
- Autoimmune Neurological Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Mayo Clinic
- National Institutes of Health
- National Institute of Neurological Disorders and Stroke