Forschung & Innovation
Publikationen
Clinical presentation, MR imaging and outcome in children with myelin oligodendrocyte glycoprotein antibody-negative acute disseminated encephalomyelitis
PMU Autor*in
G. Kluger
Alle Autor*innen
M. Panagi, A. Blaschek, A. Selek, M. Baumann, R. Cleaveland, A. Panzer, C. Conrad, A. Della Marina, K. Egler, J. Finsterwalder, T. Geis, H. Hartmann, M. Hausler, P. Hofstetter, M. Karenfort, G. Kluger, S. Leiz, A. Merkenschlager, M. Nosadini, S. Sartori, M. Salandin, M. Schimmel, B. Kornek, E. M. Wendel, M. Reindl, K. Rostasy,
Fachzeitschrift
EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY
Kurzfassung
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 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 <2) in 36/38 (94.7 %) children with MOG-neg ADEM Conclusion: A substantial number of children initially diagnosed with MOG-neg ADEM will have another diagnosis. Children with MOG-neg ADEM showed white matter lesions but also atypical MRI findings. Monophasic MOG-neg ADEM was associated with a favorable outcome.
Keywords
CENTRAL-NERVOUS-SYSTEM, MULTIPLE-SCLEROSIS, DEMYELINATION, Hemophagocytic lymphohistiocytosis