{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:37:52Z","timestamp":1760060272436,"version":"build-2065373602"},"reference-count":20,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2025,8,10]],"date-time":"2025-08-10T00:00:00Z","timestamp":1754784000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we present a case of 50-year-old female patient with long-term asthma treatment who presented initially with extreme eosinophilia (56%) and severe progressive ascending paresis, similar to Guillain\u2013Barr\u00e9 syndrome, leading to tetraplegia. After navigating through diagnostic mazes, the diagnosis of EGPA was established based on eosinophilia, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) positivity, asthma, eosinophil granulomatosis in the gastrointestinal tract, and severe peripheral nervous system involvement, complicated with rare central nervous granulomas and ischemia. With combined immunosuppressive and immunomodulatory treatment including high-dose corticosteroids, rituximab and intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation over 6 months, our patient recovered gradually from tetraplegia and adverse events such as severe infections and osteoporotic fractures. Now, from a 2-year perspective, we can conclude a successful treatment leading to decrease in all of her symptoms. Due to persistent eosinophilia after steroid tapering, she was switched to mepolizumab maintenance treatment and demonstrated continuous improvement of motor and sensory functions. Thanks to periodically repeated rehabilitation, she became self-sufficient and returned to her previous job. Our case highlights that EGPA patients should be treated in a center of expertise due to the rarity of the disease and complexity of diagnosis and treatment. Careful multidisciplinary cooperation, the huge effort of the patient, and a supportive environment can show a way back from immune-mediated tetraplegia.<\/jats:p>","DOI":"10.3390\/jcm14165652","type":"journal-article","created":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T09:59:13Z","timestamp":1754906353000},"page":"5652","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Way Back from Tetraplegia or, Rare Neurological Manifestations of Eosinophil Granulomatosis with Polyangiitis"],"prefix":"10.3390","volume":"14","author":[{"given":"Yasamin","family":"Ranjbar","sequence":"first","affiliation":[{"name":"Division of Immunology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0096-8295","authenticated-orcid":false,"given":"Tam\u00e1s","family":"\u00c1roksz\u00e1ll\u00e1si","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary"},{"name":"Doctoral School of Neuroscience, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dorottya","family":"Szinay","sequence":"additional","affiliation":[{"name":"Division of Immunology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary"},{"name":"Gyula Petr\u00e1nyi Doctoral School of Allergy and Clinical Immunology, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edit B.","family":"Nagy","sequence":"additional","affiliation":[{"name":"Doctoral School of Neuroscience, University of Debrecen, 4032 Debrecen, Hungary"},{"name":"Division of Radiology and Imaging Science, Department of Medical Imaging, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T\u00fcnde","family":"Tarr","sequence":"additional","affiliation":[{"name":"Division of Immunology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary"},{"name":"Gyula Petr\u00e1nyi Doctoral School of Allergy and Clinical Immunology, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melinda","family":"Nagy-Vincze","sequence":"additional","affiliation":[{"name":"Division of Immunology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary"},{"name":"Gyula Petr\u00e1nyi Doctoral School of Allergy and Clinical Immunology, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,8,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1093\/rheumatology\/kez570","article-title":"Eosinophilic granulomatosis with polyangiitis: Understanding the disease and its management","volume":"59","author":"Trivioli","year":"2020","journal-title":"Rheumatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"103219","DOI":"10.1016\/j.autrev.2022.103219","article-title":"Eosinophilic granulomatosis with polyangiitis: A review","volume":"22","author":"White","year":"2023","journal-title":"Autoimmun Rev."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1016\/B978-0-323-98818-6.00008-X","article-title":"Adult and childhood vasculitis","volume":"195","author":"Younger","year":"2023","journal-title":"Handb. Clin. Neurol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1002\/art.37721","article-title":"Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort","volume":"65","author":"Comarmond","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1186\/s13023-021-01780-x","article-title":"Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: A retrospective cohort study in China","volume":"16","author":"Liu","year":"2021","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1016\/j.autrev.2016.09.018","article-title":"Central nervous system involvement in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Report of 26 patients and review of the literature","volume":"16","author":"Cottin","year":"2017","journal-title":"Autoimmun. Rev."},{"key":"ref_7","first-page":"1958","article-title":"Diverse clinical manifestation of EGPA: A systematic review","volume":"83","author":"Rowell","year":"2024","journal-title":"Ann. Rheum. Dis."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/j.jaut.2013.03.003","article-title":"Longterm outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials","volume":"43","author":"Samson","year":"2013","journal-title":"J. Autoimmun."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Menon, S.G., Hugenberg, S., Alkashash, A.M., Lin, J., and Iranmanesh, A.M. (2023). Colitis as the Initial Presentation of Eosinophilic Granulomatosis with Polyangiitis. Case Rep. Rheumatol., 6620826.","DOI":"10.1155\/2023\/6620826"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"168","DOI":"10.5607\/en.2017.26.3.168","article-title":"Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis","volume":"26","author":"Yoo","year":"2017","journal-title":"Exp. Neurobiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"104036","DOI":"10.1016\/j.lpm.2020.104036","article-title":"Updates for the treatment of EGPA","volume":"49","author":"Raffray","year":"2020","journal-title":"Presse. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1016\/j.ejim.2015.04.022","article-title":"Eosinophilic granulomatosis with polyangiitis (Churg\u2013Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management","volume":"26","author":"Groh","year":"2015","journal-title":"Eur. J. Intern. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1136\/ard-2022-223764","article-title":"EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update","volume":"83","author":"Hellmich","year":"2024","journal-title":"Ann. Rheum. Dis."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.ejim.2024.06.008","article-title":"Red flags for clinical suspicion of eosinophilic granulomatosis with polyangiitis (EGPA)","volume":"128","author":"Aparicio","year":"2024","journal-title":"Eur. J. Intern. Med."},{"key":"ref_15","first-page":"981439","article-title":"Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome","volume":"2015","year":"2015","journal-title":"Case Rep. Neurol. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1186\/s41983-021-00411-3","article-title":"Eosinophilic granulomatosis with polyangiitis (Churg\u2013Strauss syndrome) imitating Guillain\u2013Barre syndrome (GBS): A case report","volume":"57","author":"Khandelwal","year":"2021","journal-title":"Egypt. J. Neurol. Psychiatry Neurosurg."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1136\/annrheumdis-2021-221794","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis","volume":"81","author":"Grayson","year":"2022","journal-title":"Ann. Rheum. Dis."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1002\/acr2.11263","article-title":"Vasculitis clinical research consortium. clinical manifestations and long-term outcomes of eosinophilic granulomatosis with polyangiitis in North America","volume":"3","author":"Doubelt","year":"2021","journal-title":"ACR Open Rheumatol."},{"key":"ref_19","first-page":"1249","article-title":"Burden of illness and costs associated with eosinophilic granulomatosis with polyangiitis: Evidence from a managed care database in the United States","volume":"27","author":"Bell","year":"2021","journal-title":"J. Manag. Care Spec. Pharm."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e6083","DOI":"10.1097\/MD.0000000000006083","article-title":"Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity","volume":"96","author":"Fraile","year":"2017","journal-title":"Medicine"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/16\/5652\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:27:29Z","timestamp":1760034449000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/16\/5652"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8,10]]},"references-count":20,"journal-issue":{"issue":"16","published-online":{"date-parts":[[2025,8]]}},"alternative-id":["jcm14165652"],"URL":"https:\/\/doi.org\/10.3390\/jcm14165652","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2025,8,10]]}}}