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Myelin Oligodendrocyte Glycoprotein and Aquaporin 4 Antibody Related Diseases In Childhood: A Nationwide Multicentric Retrospective Study

Objective: Knowledge about neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has been evolving. MOGAD usually presents with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON) and transverse myelitis (TM) and NMOSD, with TM, ON and limited forms of the disease. We assessed clinical aspects, response to treatment, outcome of these diseases. Methods: Data obtained from hospital registry systems of 13 participating centers were classified, and results displayed by descriptive statistics. Results: There were 112 patients (70 F, 63%): 79 (70%) MOGAD (MOG IgG-positive) and 33 (30%) NMOSD (aquaporin-4 IgG-positive and double negative), mean age 11.5±4.6 in MOGAD, 14.1±4.9 in NMOSD. Course was polyphasic in 33/79 (42%) MOGAD, with annualized relapse rate (ARR) 1.01±0.79 (0.3-4, median 0.8), in 17/33 (51.5%) NMOSD with ARR 0.84±0.61 (0.2-2, median 0.6). MOGAD mostly presented with ADEM (23/46, 50%) and NMOSD, with ON and/or TM (16/17, 94%). Aquaporin 4 antibody was positive in 16/33 (48.5%) of NMOSD patients. Besides typical ADEM and ON, magnetic resonance imaging (MRI) could show atypical findings: meningoencephalitis-, leukodystrophy-, or MS-like lesions in MOGAD. Sequelae, mostly visual, were observed in 21.5% MOGAD and 42.5% NMOSD cases. Frequent maintenance treatments were intravenous immunoglobulin (IVIg) in MOGAD (17/79, 21.5%), azathioprine in NMOSD (17/33, 51%). Remission was achieved with IVIg in 6/13 MOGAD and rituximab in 3/5 NMOSD. Conclusion: MOGAD was twice as frequent as NMOSD in our cohort. NMOSD patients were older. As treatment and course vary between diagnostic groups, differences in clinical presentation and outcome may assist correct classification.

İsmail Solmaz
University of Health Sciences, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital
Turkey

İbrahim Öncel
Hacettepe University Faculty of Medicine, Ihsan Dogramaci Children's Hospital
Turkey

Bahadir Konuskan
University of Health Sciences, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital
Turkey

Sanem Yılmaz
Ege University Faculty of Medicine
Turkey

İlknur Erol
Baskent University Faculty of Medicine
Turkey

Uluc Yiş
Dokuz Eylül University Faculty of Medicine
Turkey

Unsal Yilmaz
Izmir Dr Behçet Uz Children's Hospital
Turkey

Bülent Kara
Kocaeli University Faculty of Medicine
Turkey

Esra Gürkaş
Ankara Bilkent City Hospital
Turkey

Nihal Olgaç Dündar
İzmir Katip Çelebi University Faculty of Medicine
Turkey

Dilek Yalnızoğlu
Hacettepe University Faculty of Medicine Ihsan Dogramaci Children's Hospital
Turkey

Göknur Haliloglu
Hacettepe University Faculty of Medicine Ihsan Dogramaci Children's Hospital
Turkey

Aycan Unalp
İzmir Dr. Behcet Uz Children's Hospital
Turkey

Gülen Gül Mert
Çukurova University, Faculty of Medicine
Turkey

Seda Kanmaz
Ege University Faculty of Medicine
Turkey

Gamze Uzan Sarıkaya
Pediatric Neurology
Turkey

Ergin Atasoy
University of Health Sciences, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital
Turkey

Leman Tekin Orgun
Baskent University Faculty of Medicine
Turkey

Şeyda Beşen
Baskent University Faculty of Medicine
Turkey

Ayşe Aksoy
Ondokuz Mayıs University Faculty of Medicine
Turkey

Ömer Karaca
Kocaeli University Faculty of Medicine
Turkey

Şenay Haspolat
Akdeniz University Faculty of Medicine
Turkey

Hasan Tekgül
Ege University Faculty of Medicine
Turkey

Banu Anlar
Hacettepe University Faculty of Medicine Ihsan Dogramacı Children's Hospital
Turkey

 


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