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Multicenter Study
. 2023 Nov:47:118-130.
doi: 10.1016/j.ejpn.2023.10.005. Epub 2023 Oct 20.

Autoantibody status, neuroradiological and clinical findings in children with acute cerebellitis

L Quack  1 , S Glatter  2 , A Wegener-Panzer  3 , R Cleaveland  3 , A Bertolini  1 , V Endmayr  4 , R Seidl  5 , M Breu  6 , E Wendel  7 , M Schimmel  8 , M Baumann  9 , M Rauchenzauner  10 , M Pritsch  11 , N Boy  12 , T Muralter  13 , G Kluger  14 , C Makoswski  15 , V Kraus  16 , S Leiz  17 , C Loehr-Nilles  18 , J H Kreth  19 , S Braig  20 , S Schilling  21 , J Kern  22 , C Blank  23 , B Tro Baumann  11 , S Vieth  24 , M Wallot  5 , M Reindl  25 , H Ringl  26 , K P Wandinger  27 , F Leypoldt  28 , R Höftberger  29 , K Rostásy  30
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Free article
Multicenter Study

Autoantibody status, neuroradiological and clinical findings in children with acute cerebellitis

L Quack et al. Eur J Paediatr Neurol. 2023 Nov.
Free article

Erratum in

Abstract

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.

Keywords: Autoimmune; Cerebellitis; Children; MOG antibodies.

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Conflict of interest statement

Declaration of competing interest L. Quack: no. S. Glatter: no. A. Wegener-Panzer: no. B. Cleaveland: no. A. Bertolini: no. V. Endmayr: R. Seidl:no. E. Wendel: no. M. Schimmel: no. M. Baumann: M. Rauchenzauner: M. Pritsch: no. N. Boy: no. T. Muralter: no. G. Kluger:no. V. Kraus: no. S. Leiz:no. C. Loehr-Nilles: no. J.H. Kreth: no. S. Braig: no. S. Schilling: no. J. Kern: no. C. Blank: no. B. Tro Baumann: no. S. Vieth: no. M. Wallot: no. H. Ringl: no. C. Makoswski: speakers honoraria from Desitin, Esai, Danone, Roche. M. Breu: speaker honoraria from Sanofi Genzyme. M Reindl was supported by a research support from Euroimmun and Roche. The University Hospital and Medical University of Innsbruck (Austria, employer of Dr. Reindl) receives payments for antibody assays (MOG, AQP4, and other autoantibodies) and for MOG and AQP4 antibody validation experiments organized by Euroimmun (Lübeck, Germany). F. Leypoldt: Reports speaker's honoraria from Grifols, Biogen, Bayer, Roche and Teva, advisory board positions for Roche and Biogen. FL's institute performs commercial antibody testing without FL receiving any personal financial benefit. Höftberger: speaker's honoraria from Novartis and Biogen. The Medical University of Vienna (Austria; employer of Dr. Höftberger) receives payment for antibody assays and for antibody validation experiments organized by Euroimmun (Lübeck, Germany). K. Rostasy: Serves as consultant for Roche in Operetta II trial and received speaker's honoraria from Merck.

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