Clinical presentation, MR imaging and outcome in children with myelin oligodendrocyte glycoprotein antibody-negative acute disseminated encephalomyelitis

Panagi, M.; Blaschek, A.; Selek, A.; Baumann, M.; Cleaveland, R.; Panzer, A.; Conrad, C.; Della Marina, A.; Egler, K.; Finsterwalder, J.; Geis, T.; Hartmann, H.; Häusler, M.; Hofstetter, P.; Karenfort, M.; Kluger, G.; Leiz, S.; Merkenschlager, A.; Nosadini, M.; Sartori, S.; Salandin, M.; Schimmel, M.; Kornek, B.; Wendel, E.M.; Reindl, M.; Rostasy, K.

doi:10.1016/j.ejpn.2025.05.009

Clinical presentation, MR imaging and outcome in children with myelin oligodendrocyte glycoprotein antibody-negative acute disseminated encephalomyelitis

Panagi M., Blaschek A., Selek A., Baumann M., Cleaveland R., Panzer A., ...Daha Fazla

European Journal of Paediatric Neurology, cilt.57, ss.24-34, 2025 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 57
Basım Tarihi: 2025
Doi Numarası: 10.1016/j.ejpn.2025.05.009
Dergi Adı: European Journal of Paediatric Neurology
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE
Sayfa Sayıları: ss.24-34
Anahtar Kelimeler: (1) acute disseminated encephalomyelitis (ADEM), (2) children, (3) myelin oligodendrocyte glycoprotein (MOG), (4) multiple sclerosis (MS), (5) HLH
Sağlık Bilimleri Üniversitesi Adresli: Hayır

Özet

Background: Acute disseminated encephalomyelitis (ADEM) without myelin oligodendrocyte glycoprotein (MOG) antibodies (abs) presents a diagnostic challenge. Objective: To investigate whether the diagnosis of MOG-negative (neg) ADEM was confirmed over time and to highlight the clinical and neuroradiological characteristics distinguishing monophasic MOG-neg ADEM from alternative diagnoses. Material and methods: Children diagnosed with a first clinical episode of MOG-neg ADEM and a dataset including clinical presentation, MRI, CSF studies and at least 3-month follow-up were included. Results: 47 children with MOG-neg ADEM were identified (m:f = 27:20, median age 8.0 years. 38 (79.2 %) children maintained the initial diagnosis after a median follow-up of 34.2 months. In 9 (19.1 %) children an alternative diagnosis was assigned after a median follow-up of 4.2 months including multiple sclerosis (MS) n = 2; glioblastoma (GBM) n = 2; hemophagocytic lymphohistiocytosis (HLH) n = 2; CNS vasculitis n = 1; ADEM followed by optic neuritis (ADEMON) n = 2. Cerebral white matter lesions were identified in 84.2 % of MOG-neg ADEM children. Gadolinium enhancement was noted in 11.4 % of MOG-neg ADEM children. Of 38 MOG-neg ADEM children, 9 (23.7 %) had only one atypical MRI finding, whereas 23 (60.5 %) showed multiple atypical MRI features. All children with alternative diagnoses exhibited more than one atypical MRI feature. The outcome was favorable (mRS