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Given the fatal prognosis of LMND, identifying underlying autoimmune syndromes is crucial to provide treatment options to patients.<\/jats:p>\n                  <jats:p>We investigated a novel autoantibody binding pattern observed on murine teased sciatic nerve fibres. Target antigens were identified using immunoprecipitation combined with mass spectrometry. Target specificity of these autoantibodies was validated in cell-based assays, neutralization assays and knock-out models. A retrospective study cohort consisting of different neuropathies (chronic inflammatory demyelinating polyradiculopathy n = 86, Guillain\u2013Barr\u00e9 syndrome n = 37, multifocal motor neuropathy n = 18, diabetic neuropathy n = 30, other inflammatory neuropathies n = 10), amyotrophic lateral sclerosis (n = 50), multiple sclerosis (n = 50) and healthy controls (n = 50) was negative for septin multimer autoantibodies. Histopathological analysis of skin and the sural nerve including electron microscopy was performed in one seropositive patient, and autoantibody binding was characterized in vitro. Extensive immunotherapy was initiated in one patient, with clinical and serological follow-up over 4 years.<\/jats:p>\n                  <jats:p>Among 3543 total samples tested, three patients (two male, one female)\u2014diagnosed with the LMND variant of amyotrophic lateral sclerosis (aged 65, 72 and 79 years, respectively)\u2014showed a novel and distinct autoantibody binding pattern of indirect immunofluorescence staining on peripheral nerves, targeting Schmidt\u2013Lanterman incisures (SLIs), paranodes and the abaxonal myelin. Target identification and validation revealed septin multimers as autoantibody epitopes. Despite the primarily intracellular location of septins, autoantibody binding was evident in living myelinated dorsal root ganglia, primarily at SLIs (\u2018incisuropathy\u2019). Septin multimer autoantibodies further initiated complement deposition on fixed and permeabilized cell-based assays. Sural nerve and skin biopsies showed inflammation, myelin and axonal pathology. Extensive immunotherapy in one patient was followed by disease stabilization over 3 years. The other two patients died of rapid disease progression: one of them received no immunotherapy while the other had ineffective treatments with single administrations of intravenous immunoglobulin and rituximab.<\/jats:p>\n                  <jats:p>Our data suggest that septin multimer autoimmunity occurs in severe motor-predominant neuropathies which can clinically resemble a neurodegenerative LMND. Screening for septin multimer autoantibodies should be considered in patients presenting with this phenotype. Follow-up studies need to determine the direct pathogenicity of septin multimer autoantibodies, their potential as a biomarker of an autoimmune syndrome and responses to immunotherapy in larger cohorts.<\/jats:p>","DOI":"10.1093\/brain\/awag183","type":"journal-article","created":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T11:47:05Z","timestamp":1780055225000},"page":"2731-2746","source":"Crossref","is-referenced-by-count":0,"title":["Septin multimer autoantibodies in severe motor neuropathy mimicking lower motor neuron disease"],"prefix":"10.1093","volume":"149","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0112-8685","authenticated-orcid":false,"given":"Friederike A","family":"Arlt","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, MN 55901 ,","place":["USA"]},{"name":"Department of Neurology and Experimental Neurology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]},{"name":"German Center for Neurodegenerative Diseases (DZNE) Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramona","family":"Miske","sequence":"additional","affiliation":[{"name":"Institute for Experimental Immunology, affiliated with EUROIMMUN Medizinische Labordiagnostika AG , L\u00fcbeck 23560 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5248-4009","authenticated-orcid":false,"given":"Luise","family":"Appeltshauser","sequence":"additional","affiliation":[{"name":"Department of Neurology and Experimental Neurology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]},{"name":"German Center for Neurodegenerative Diseases (DZNE) Berlin , Berlin 10117 ,","place":["Germany"]},{"name":"Department of Neurology, University Hospital W\u00fcrzburg , W\u00fcrzburg 97080 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viktoria","family":"Zinnow","sequence":"additional","affiliation":[{"name":"Department of Neurology and Experimental Neurology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]},{"name":"German Center for Neurodegenerative Diseases (DZNE) Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Borowski","sequence":"additional","affiliation":[{"name":"Clinical Immunological Laboratory Prof.h.c. (RCH) Dr.med. Winfried St\u00f6cker , L\u00fcbeck 23627 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner","family":"Stenzel","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helena","family":"Radbruch","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7233-5342","authenticated-orcid":false,"given":"Andreas","family":"Meisel","sequence":"additional","affiliation":[{"name":"Department of Neurology and Experimental 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,","place":["Germany"]},{"name":"German Center for Neurodegenerative Diseases (DZNE) Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah","family":"Pressler","sequence":"additional","affiliation":[{"name":"Department of Neurology and Experimental Neurology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]},{"name":"German Center for Neurodegenerative Diseases (DZNE) Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5306-7880","authenticated-orcid":false,"given":"Frauke","family":"Stascheit","sequence":"additional","affiliation":[{"name":"Department of Neurology and Experimental Neurology, Charit\u00e9-Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t and Humboldt-Universit\u00e4t zu Berlin , Berlin 10117 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janis","family":"Linke","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital W\u00fcrzburg , W\u00fcrzburg 97080 ,","place":["Germany"]},{"name":"Rudolf Virchow Center, Center for Integrative and Translational Bioimaging, Julius-Maximilians-Universit\u00e4t W\u00fcrzburg (JMU) , W\u00fcrzburg 97080 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"H\u00fcmmert","sequence":"additional","affiliation":[{"name":"Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences , G\u00f6ttingen 37077 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hauke B","family":"Werner","sequence":"additional","affiliation":[{"name":"Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences , G\u00f6ttingen 37077 ,","place":["Germany"]},{"name":"Faculty for Biology and Psychology, University of G\u00f6ttingen , G\u00f6ttingen 37073 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esravila A","family":"Wibisono","sequence":"additional","affiliation":[{"name":"Internal Medicine, Saint Mary's Hospital , Waterbury, CT 06706 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Doppler","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital W\u00fcrzburg , W\u00fcrzburg 97080 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Komorowski","sequence":"additional","affiliation":[{"name":"Institute for Experimental Immunology, affiliated with EUROIMMUN Medizinische Labordiagnostika AG , L\u00fcbeck 23560 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elias T","family":"Spiliotis","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, University of Virginia School of Medicine , Charlottesville, VA 22903 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Divyanshu","family":"Dubey","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, MN 55901 ,","place":["USA"]},{"name":"Department of Neurology, Center MS and Autoimmune Neurology, Mayo Clinic , Rochester, MN 55901 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia","family":"Zeckeridou","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, MN 55901 ,","place":["USA"]},{"name":"Department of Neurology, Center MS and Autoimmune Neurology, Mayo Clinic , Rochester, MN 55901 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