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Serum samples were collected for TBA. The predictors of relapse were examined over a minimum of 24\u2009months follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In our cohort of 98 patients with double negative atypical IDD, there was no significant female predominance (58.2%, 57\/98). The lesions primarily affected the spinal cord and brain stem, with fewer cases of involvement in the area postrema (5.1%, 5\/98) and longitudinally extensive transverse myelitis (43.9%, 43\/98). A total of 62.5% (50\/80) patients tested positive for anti\u2010astrocyte antibodies based on rat brain TBA. Over a median duration of 39.5\u2009months, 80 patients completed the entire follow\u2010up, and 47.5% (38\/80) patients exhibited monophasic course. A total of 36% (18\/50) patients positively for anti\u2010astrocyte antibodies had a monophasic course, which is significantly lower than patients negatively for anti\u2010astrocyte antibodies (66.7%, 20\/30) (<jats:italic>p<\/jats:italic>\u2009=\u20090.008). The presence of anti\u2010astrocyte antibodies (hazard ratio (HR), 2.243; 95% CI, 1.087\u20134.627; <jats:italic>p<\/jats:italic>\u2009=\u20090.029) and\u2009\u22654 cerebrum lesions at first attack (HR, 2.494; 95% CI, 1.224\u20135.078; <jats:italic>p<\/jats:italic>\u2009=\u20090.012) were risk factors for disease relapse, while maintenance immunotherapy during remission (HR, 0.361; 95% CI, 0.150\u20130.869; <jats:italic>p<\/jats:italic>\u2009=\u20090.023) was protective factor.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Double negative atypical IDD are unique demyelinating diseases with a high relapse rate. Maintenance immunotherapy is helpful to the prevention of relapse, particularly in patients with anti\u2010astrocyte antibodies or \u22654 cerebrum lesions at first attack.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.52191","type":"journal-article","created":{"date-parts":[[2024,9,2]],"date-time":"2024-09-02T18:04:34Z","timestamp":1725300274000},"page":"2769-2784","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Clinical characteristics of double negative atypical inflammatory demyelinating disease: A prospective study"],"prefix":"10.1002","volume":"11","author":[{"given":"Fei","family":"Jiang","sequence":"first","affiliation":[{"name":"Department of Neurology, Xiangya Hospital Central South University Changsha 410000 Hunan P.R. China"},{"name":"Clinical Research Center for Neuroimmune and Neuromuscular disorders, Xiangya Hospital Central South University Changsha 410008 Hunan P.R. China"},{"name":"National Clinical Research Center for Geriatric Disorders, Xiangya Hospital Central South University Changsha 410008 Hunan P.R. China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haobing","family":"Cai","sequence":"additional","affiliation":[{"name":"Department of Neurology, Xiangya Hospital Central South University Changsha 410000 Hunan P.R. China"},{"name":"Clinical Research Center for Neuroimmune and Neuromuscular disorders, Xiangya Hospital Central South University Changsha 410008 Hunan P.R. China"},{"name":"National Clinical Research Center for Geriatric Disorders, Xiangya Hospital Central South University Changsha 410008 Hunan P.R. China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongliang","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Acupuncture and Tuina Rehabilitation The First Hospital of Hunan University of Chinese Medicine Changsha 410000 Hunan P.R. 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