{"title":"改良拉链法治疗类固醇抵抗性ADEM 1例。","authors":"Nihal Akçay, İlyas Bingöl, Hilal Merve Dolu, Behice Kaniye Yılmaz, Demet Tosun","doi":"10.1007/s13760-025-02788-x","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Acute disseminated encephalomyelitis (ADEM) is a rare, inflammatory, demyelinating central nervous system disorder typically responsive to corticosteroid therapy. However, some cases are resistant to steroids, requiring alternative treatment options.</p><p><strong>Case presentation: </strong>We present an 8-year-old male child with steroid-resistant ADEM who underwent treatment with the modified zipper technique, combining intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Despite initial high-dose intravenous methylprednisolone, which failed to improve his condition, the patient showed significant clinical and radiological improvement after five cycles of the modified zipper technique.</p><p><strong>Methods: </strong>The patient received alternating cycles of TPE and IVIG (0.4 g/kg/day) over five cycles, with a 48-hour interval between each TPE and IVIG session. TPE sessions used fresh frozen plasma, followed by IVIG to enhance antibody clearance and immunomodulation.</p><p><strong>Results: </strong>After two cycles of TPE, clinical improvement was observed. Follow-up MRI on day 15 showed marked regression of white matter lesions. The patient was discharged on day 20 with near-complete recovery. Follow-up at one, three, and six months showed no neurological deficits, and MRI lesions continued to regress.</p><p><strong>Conclusions: </strong>This case suggests the modified zipper technique as a promising alternative for steroid-resistant ADEM. The combination of TPE and IVIG may provide beneficial effects, but further controlled studies are needed to evaluate its efficacy and safety in broader populations.</p>","PeriodicalId":7042,"journal":{"name":"Acta neurologica Belgica","volume":" ","pages":""},"PeriodicalIF":2.0000,"publicationDate":"2025-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Modified zipper method in steroid-resistant ADEM: a case report.\",\"authors\":\"Nihal Akçay, İlyas Bingöl, Hilal Merve Dolu, Behice Kaniye Yılmaz, Demet Tosun\",\"doi\":\"10.1007/s13760-025-02788-x\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Acute disseminated encephalomyelitis (ADEM) is a rare, inflammatory, demyelinating central nervous system disorder typically responsive to corticosteroid therapy. However, some cases are resistant to steroids, requiring alternative treatment options.</p><p><strong>Case presentation: </strong>We present an 8-year-old male child with steroid-resistant ADEM who underwent treatment with the modified zipper technique, combining intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Despite initial high-dose intravenous methylprednisolone, which failed to improve his condition, the patient showed significant clinical and radiological improvement after five cycles of the modified zipper technique.</p><p><strong>Methods: </strong>The patient received alternating cycles of TPE and IVIG (0.4 g/kg/day) over five cycles, with a 48-hour interval between each TPE and IVIG session. TPE sessions used fresh frozen plasma, followed by IVIG to enhance antibody clearance and immunomodulation.</p><p><strong>Results: </strong>After two cycles of TPE, clinical improvement was observed. Follow-up MRI on day 15 showed marked regression of white matter lesions. The patient was discharged on day 20 with near-complete recovery. Follow-up at one, three, and six months showed no neurological deficits, and MRI lesions continued to regress.</p><p><strong>Conclusions: </strong>This case suggests the modified zipper technique as a promising alternative for steroid-resistant ADEM. The combination of TPE and IVIG may provide beneficial effects, but further controlled studies are needed to evaluate its efficacy and safety in broader populations.</p>\",\"PeriodicalId\":7042,\"journal\":{\"name\":\"Acta neurologica Belgica\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":2.0000,\"publicationDate\":\"2025-04-16\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta neurologica Belgica\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s13760-025-02788-x\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta neurologica Belgica","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s13760-025-02788-x","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Modified zipper method in steroid-resistant ADEM: a case report.
Background: Acute disseminated encephalomyelitis (ADEM) is a rare, inflammatory, demyelinating central nervous system disorder typically responsive to corticosteroid therapy. However, some cases are resistant to steroids, requiring alternative treatment options.
Case presentation: We present an 8-year-old male child with steroid-resistant ADEM who underwent treatment with the modified zipper technique, combining intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Despite initial high-dose intravenous methylprednisolone, which failed to improve his condition, the patient showed significant clinical and radiological improvement after five cycles of the modified zipper technique.
Methods: The patient received alternating cycles of TPE and IVIG (0.4 g/kg/day) over five cycles, with a 48-hour interval between each TPE and IVIG session. TPE sessions used fresh frozen plasma, followed by IVIG to enhance antibody clearance and immunomodulation.
Results: After two cycles of TPE, clinical improvement was observed. Follow-up MRI on day 15 showed marked regression of white matter lesions. The patient was discharged on day 20 with near-complete recovery. Follow-up at one, three, and six months showed no neurological deficits, and MRI lesions continued to regress.
Conclusions: This case suggests the modified zipper technique as a promising alternative for steroid-resistant ADEM. The combination of TPE and IVIG may provide beneficial effects, but further controlled studies are needed to evaluate its efficacy and safety in broader populations.
期刊介绍:
Peer-reviewed and published quarterly, Acta Neurologica Belgicapresents original articles in the clinical and basic neurosciences, and also reports the proceedings and the abstracts of the scientific meetings of the different partner societies. The contents include commentaries, editorials, review articles, case reports, neuro-images of interest, book reviews and letters to the editor.
Acta Neurologica Belgica is the official journal of the following national societies:
Belgian Neurological Society
Belgian Society for Neuroscience
Belgian Society of Clinical Neurophysiology
Belgian Pediatric Neurology Society
Belgian Study Group of Multiple Sclerosis
Belgian Stroke Council
Belgian Headache Society
Belgian Study Group of Neuropathology