Pediatric-Onset Chronic Inflammatory Demyelinating Polyneuropathy: A Multicenter Study - 05/07/23
, Atay Vural, MD b, c : Associate Professor, Deniz Yüksel, MD d : Professor, Erhan Aksoy, MD d : Associate Professor, Ülkühan Öztoprak, MD d : Associate Professor, Mehmet Canpolat, MD e : Professor, Selcan Öztürk, MD e, Çelebi Yıldırım, MD f, Ayten Güleç, MD e, Hüseyin Per, MD e : Professor, Hakan Gümüş, MD e : Professor, Çetin Okuyaz, MD g : Professor, Meltem Çobanoğulları Direk, MD g : Associate Professor, Mustafa Kömür, MD g : Associate Professor, Aycan Ünalp, MD h : Professor, Ünsal Yılmaz, MD h : Professor, Ömer Bektaş, MD i : Associate Professor, Serap Teber, MD i : Professor, Nargiz Aliyeva, MD j, Nihal Olgaç Dündar, MD k : Professor, Pınar Gençpınar, MD k : Professor, Esra Gürkaş, MD l : Professor, Sanem Keskin Yılmaz, MD m : Associate Professor, Seda Kanmaz, MD m, Hasan Tekgül, MD m : Professor, Ayşe Aksoy, MD n : Professor, Gökçen Öz Tuncer, MD n, Elif Acar Arslan, MD o : Associate Professor, Ayşe Tosun, MD p : Professor, Müge Ayanoğlu, MD p : Associate Professor, Ali Burak Kızılırmak b : PhD Candidate, Mohammadreza Yousefi b : PhD Candidate, Muhittin Bodur, MD q, Bülent Ünay, MD r : Professor, Semra Hız Kurul, MD a : Professor, Uluç Yiş, MD a : ProfessorAbstract |
Background |
To evaluate the clinical features, demographic features, and treatment modalities of pediatric-onset chronic inflammatory demyelinating polyneuropathy (CIDP) in Turkey.
Methods |
The clinical data of patients between January 2010 and December 2021 were reviewed retrospectively. The patients were evaluated according to the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society Guideline on the management of CIDP (2021). In addition, patients with typical CIDP were divided into two groups according to the first-line treatment modalities (group 1: IVIg only, group 2: IVIg + steroid). The patients were further divided into two separate groups based on their magnetic resonance imaging (MRI) characteristics.
Results |
A total of 43 patients, 22 (51.2%) males and 21 (48.8%) females, were included in the study. There was a significant difference between pretreatment and post-treatment modified Rankin scale (mRS) scores (P < 0.05) of all patients. First-line treatments include intravenous immunoglobulin (IVIg) (n = 19, 44.2%), IVIg + steroids (n = 20, 46.5%), steroids (n = 1, 2.3%), IVIg + steroids + plasmapheresis (n = 1, 2.3%), and IVIg + plasmapheresis (n = 1, 2.3%). Alternative agent therapy consisted of azathioprine (n = 5), rituximab (n = 1), and azathioprine + mycophenolate mofetil + methotrexate (n = 1). There was no difference between the pretreatment and post-treatment mRS scores of groups 1 and 2 (P > 0.05); however, a significant decrease was found in the mRS scores of both groups with treatment (P < 0.05). The patients with abnormal MRI had significantly higher pretreatment mRS scores compared with the group with normal MRI (P < 0.05).
Conclusions |
This multicenter study demonstrated that first-line immunotherapy modalities (IVIg vs IVIg + steroids) had equal efficacy for the treatment of patients with CIDP. We also determined that MRI features might be associated with profound clinical features, but did not affect treatment response.
Le texte complet de cet article est disponible en PDF.Keywords : Chronic inflammatory demyelinating polyneuropathy, Childhood, Immunotherapy, IVIg, Steroids, Neurofascin antibodies
Plan
| Funding: This study was funded by The Scientific and Technological Research Council of Turkey, Grant No.: 118S397. |
Vol 145
P. 3-10 - août 2023 Retour au numéro
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