Autoantibody status, neuroradiological and clinical findings in children with acute cerebellitis
Background - Acute cerebellitis (AC) in children and adolescents is an inflammatory disease of the cerebellum due to viral or bacterial infections but also autoimmune-mediated processes. - Objective - To investigate the frequency of autoantibodies in serum and CSF as well as the neuroradiological fe...
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| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
November 2023
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| In: |
European journal of paediatric neurology
Year: 2023, Volume: 47, Pages: 118-130 |
| ISSN: | 1532-2130 |
| DOI: | 10.1016/j.ejpn.2023.10.005 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.ejpn.2023.10.005 Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1090379823001605 
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| Author Notes: | L. Quack, S. Glatter, A. Wegener-Panzer, R. Cleaveland, A. Bertolini, V. Endmayr, R. Seidl, M. Breu, E. Wendel, M. Schimmel, M. Baumann, M. Rauchenzauner, M. Pritsch, N. Boy, T. Muralter, G. Kluger, C. Makoswski, V. Kraus, S. Leiz, C. Loehr-Nilles, J. H. Kreth, S. Braig, S. Schilling, J. Kern, C. Blank, B. Tro Baumann, S. Vieth, M. Wallot, M. Reindl, H. Ringl, K. P. Wandinger, F. Leypoldt, R. Höftberger, K. Rostásy |
| Summary: | Background - Acute cerebellitis (AC) in children and adolescents is an inflammatory disease of the cerebellum due to viral or bacterial infections but also autoimmune-mediated processes. - Objective - To investigate the frequency of autoantibodies in serum and CSF as well as the neuroradiological features in children with AC. - Material and methods - Children presenting with symptoms suggestive of AC defined as acute/subacute onset of cerebellar symptoms and MRI evidence of cerebellar inflammation or additional CSF pleocytosis, positive oligoclonal bands (OCBs), and/or presence of autoantibodies in case of negative cerebellar MRI. Children fulfilling the above-mentioned criteria and a complete data set including clinical presentation, CSF studies, testing for neuronal/cerebellar and MOG antibodies as well as MRI scans performed at disease onset were eligible for this retrospective multicenter study. - Results - 36 patients fulfilled the inclusion criteria for AC (f:m = 14:22, median age 5.5 years). Ataxia was the most common cerebellar symptom present in 30/36 (83 %) in addition to dysmetria (15/36) or dysarthria (13/36). A substantial number of children (21/36) also had signs of encephalitis such as somnolence or seizures. In 10/36 (28 %) children the following autoantibodies (abs) were found: MOG-abs (n = 5) in serum, GFAPα-abs (n = 1) in CSF, GlyR-abs (n = 1) in CSF, mGluR1-abs (n = 1) in CSF and serum. In two further children, antibodies were detected only in serum (GlyR-abs, n = 1; GFAPα-abs, n = 1). MRI signal alterations in cerebellum were found in 30/36 children (83 %). Additional supra- and/or infratentorial lesions were present in 12/36 children, including all five children with MOG-abs. Outcome after a median follow-up of 3 months (range: 1 a 75) was favorable with an mRS ≤2 in 24/36 (67 %) after therapy. Antibody (ab)-positive children were significantly more likely to have a better outcome than ab-negative children (p = .022). - Conclusion - In nearly 30 % of children in our study with AC, a range of abs was found, underscoring that autoantibody testing in serum and CSF should be included in the work-up of a child with suspected AC. The detection of MOG-abs in AC does expand the MOGAD spectrum. |
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| Item Description: | Online veröffentlicht am 20.10.2023 Gesehen am 23.04.2024 |
| Physical Description: | Online Resource |
| ISSN: | 1532-2130 |
| DOI: | 10.1016/j.ejpn.2023.10.005 |