{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T16:18:03Z","timestamp":1789402683901,"version":"build-2803163510"},"reference-count":15,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,12,28]],"date-time":"2020-12-28T00:00:00Z","timestamp":1609113600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To describe CSF\u2010defined neuronal intermediate filament (NIF) autoimmunity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>NIF\u2010IgG CSF\u2010positive patients (41, 0.03% of 118599 tested, 1996\u20132019) were included (serum was neither sensitive nor specific). Criteria\u2010based patient NIF\u2010IgG staining of brain and myenteric NIFs was detected by indirect immunofluorescence assay (IFA); NIF\u2010specificity was confirmed by cell\u2010based assays (CBAs, alpha internexin, neurofilament light [NF\u2010L]), heavy\u2010[NF\u2010H] chain).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Sixty\u2010one percent of 41 patients were men, median age, 61\u00a0years (range, 21\u201388). Syndromes were encephalopathy predominant (23), cerebellar ataxia predominant (11), or myeloradiculoneuropathies (7). MRI abnormalities (T2 hyperintensities of brain, spinal cord white matter tracts. and peripheral nerve axons) and neurophysiologic testing (EEG, EMG, evoked potentials) co\u2010localized with clinical neurological phenotypes (multifocal in 29%). Thirty patients (73%) had\u00a0\u2265\u00a01 immunological perturbation: cancer (paraneoplastic), 22; systemic infection (parainfectious [including ehrlichosis, 3] or HIV), 7; checkpoint\u2010inhibitor cancer immunotherapy, 4; other, 5. Cancers were as follows: neuroendocrine\u2010lineage carcinomas, 12 (small cell, 6; Merkel cell, 5; pancreatic, 1 [11\/12 had NF\u2010L\u2010IgG detected, versus 8\/29 others,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.0005]) and other, 11. Onset was predominantly subacute (92%) and accompanied by inflammatory CSF (75%), and immunotherapy response (77%). In contrast, CSF controls (15684 total) demonstrated NIF\u2010IgG negativity (100% of test validation controls), and low frequencies of autoimmune diagnoses (20% of consecutively referred clinical specimens) and neuroendocrine\u2010lineage carcinoma diagnosis (3.1% vs. 30% of NIF cases),\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0&lt;\u00a00.0001. Median NF\u2010L protein concentration was higher in 8 NF\u2010L\u2010IgG\u2010positive patients (median, 6718\u00a0ng\/L) than 16 controls.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Neurological autoimmunity, defined by CSF\u2010detected NIF\u2010IgGs, represents a continuum of treatable axonopathies, sometimes paraneoplastic or parainfectious.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acn3.51284","type":"journal-article","created":{"date-parts":[[2020,12,28]],"date-time":"2020-12-28T10:15:27Z","timestamp":1609150527000},"page":"425-439","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["Neuronal intermediate filament IgGs in CSF: Autoimmune Axonopathy Biomarkers"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6856-8143","authenticated-orcid":false,"given":"Andrew","family":"McKeon","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"},{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shahar","family":"Shelly","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Zivelonghi","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"},{"name":"Neurology Unit Department of Neurosciences, Biomedicine and Movement Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eati","family":"Basal","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4302-4075","authenticated-orcid":false,"given":"Divyanshu","family":"Dubey","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"},{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eoin","family":"Flanagan","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"},{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ajay A.","family":"Madhavan","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Mariotto","sequence":"additional","affiliation":[{"name":"Neurology Unit Department of Neurosciences, Biomedicine and Movement Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Toledano","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer A.","family":"Tracy","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia","family":"Zekeridou","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic 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