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

Autor: Quack L; Department of Pediatric Neurology, Childreńs Hospital Datteln, University Witten/Herdecke, Datteln, Germany., Glatter S; Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Austria; Comprehensive Center for Pediatrics, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria., Wegener-Panzer A; Department of Pediatric Radiology, Childreńs Hospital Datteln, University Witten/Herdecke, Datteln, Germany., Cleaveland R; Department of Pediatric Radiology, Childreńs Hospital Datteln, University Witten/Herdecke, Datteln, Germany., Bertolini A; Department of Pediatric Neurology, Childreńs Hospital Datteln, University Witten/Herdecke, Datteln, Germany., Endmayr V; Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Austria., Seidl R; Department of Pediatrics, Bethanien Hospital, Moers, Germany., Breu M; Division of Pediatric Pulmonology, Allergology and Endocrinology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria., Wendel E; Division of Pediatric Neurology, Department of Pediatrics, Olgahospital, Stuttgart, Germany., Schimmel M; Division of Pediatric Neurology, Clinic of Pediatrics, Augsburg University Hospital, University of Augsburg, Augsburg, Germany., Baumann M; Department of Pediatric I, Pediatric Neurology, Medical University of Innsbruck, Innsbruck, Austria., Rauchenzauner M; Department of Pediatric I, Pediatric Neurology, Medical University of Innsbruck, Innsbruck, Austria; Centre of Epilepsy for Children and Adolescents, Schoen Klinik Vogtareuth, Hospital for Neuropediatrics and Neurological Rehabilitation, Vogtareuth, Germany., Pritsch M; Department of Neuropediatrics, Children's Hospital DRK Siegen, Siegen, Germany., Boy N; Centre for Child and Adolescent Medicine, Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg, Heidelberg, Germany., Muralter T; Centre of Epilepsy for Children and Adolescents, Schoen Klinik Vogtareuth, Hospital for Neuropediatrics and Neurological Rehabilitation, Vogtareuth, Germany., Kluger G; Centre of Epilepsy for Children and Adolescents, Schoen Klinik Vogtareuth, Hospital for Neuropediatrics and Neurological Rehabilitation, Vogtareuth, Germany; Research Institute for Rehabilitation, Transition, and Palliation, Paracelsus Medical University Salzburg, Salzburg, Austria., Makoswski C; Pediatric Neurology, Department of Pediatrics, Kinderklinik München Schwabing, School of Medicine, Technical University of Munich, Germany., Kraus V; Pediatric Neurology, Department of Pediatrics, Kinderklinik München Schwabing, School of Medicine, Technical University of Munich, Germany; Social Pediatrics, Department of Pediatrics, Technical University of Munich, Munich, Germany., Leiz S; Department of Pediatrics and Adolescent Medicine, Hospital Dritter Orden, Munich, Germany., Loehr-Nilles C; Department of Neuropediatrics, Klinikum Mutterhaus der Borromäerinnen, Trier, Germany., Kreth JH; Department of Neuropediatrics, Social Pediatric Center, Klinikum Leverkusen, Leverkusen, Germany., Braig S; Department of Pediatrics, Klinikum Bayreuth, Bayreuth, Germany., Schilling S; Department of Neuropediatrics, Clinic of Pediatrics, Barmherzige Brüder St. Hedwig Hospital, Regensburg, Germany., Kern J; Department of Pediatric Neurology and Developmental Medicine, University Children's Hospital Tübingen, Germany., Blank C; Department of Pediatric Neurology, Children's Hospital St. Marien, Landshut, Germany., Tro Baumann B; Department of Neuropediatrics, Children's Hospital DRK Siegen, Siegen, Germany., Vieth S; Department of Pediatrics, University Medical Center Schleswig Holstein, Kiel, Germany., Wallot M; Department of Pediatrics, Bethanien Hospital, Moers, Germany., Reindl M; Clinical Department of Neurology, Medical University of Innsbruck, Austria., Ringl H; Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Austria; Department of Radiology, Klinik Donaustadt, Vienna, Austria., Wandinger KP; Institute of Clinical Chemistry, University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany., Leypoldt F; Institute of Clinical Chemistry, University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology, University Hospital Schleswig-Holstein, Kiel, Germany., Höftberger R; Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Austria; Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria. Electronic address: romana.hoeftberger@meduniwien.ac.at., Rostásy K; Department of Pediatric Neurology, Childreńs Hospital Datteln, University Witten/Herdecke, Datteln, Germany. Electronic address: k.rostasy@kinderklinik-datteln.de.
Jazyk: angličtina
Zdroj: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society [Eur J Paediatr Neurol] 2023 Nov; Vol. 47, pp. 118-130. Date of Electronic Publication: 2023 Oct 20.
DOI: 10.1016/j.ejpn.2023.10.005
Abstrakt: 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.
Competing Interests: 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.
(© 2023 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.)
Databáze: MEDLINE
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