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Copine-4 Paraneoplastic Autoimmune Encephalitis
Mimi S Fang1, Amy Ml Quek2,3, Clement Yong2,4
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Abstract:
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.
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