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

被引:4
|
作者
Quack, L. [1 ]
Glatter, S. [2 ,3 ]
Wegener-Panzer, A. [4 ]
Cleaveland, R. [4 ]
Bertolini, A. [1 ]
Endmayr, V. [2 ]
Seidl, R.
Breu, M. [5 ]
Wendel, E. [6 ]
Schimmel, M. [7 ]
Baumann, M. [8 ]
Rauchenzauner, M. [8 ]
Pritsch, M. [9 ]
Boy, N. [10 ]
Muralter, T. [11 ]
Kluger, G. [11 ,12 ]
Makoswski, C. [13 ]
Kraus, V. [13 ,14 ]
Leiz, S. [15 ]
Loehr-Nilles, C. [16 ]
Kreth, J. H. [17 ]
Braig, S. [18 ]
Schilling, S. [19 ]
Kern, J. [20 ]
Blank, C. [21 ]
Baumann, B. Tro [9 ]
Vieth, S. [22 ]
Wallot, M. [23 ]
Reindl, M. [24 ]
Ringl, H. [25 ,26 ]
Wandinger, K. P. [27 ]
Leypoldt, F. [27 ,28 ]
Hoeftberger, R. [2 ,29 ]
Rostasy, K. [1 ]
机构
[1] Univ Witten Herdecke, Childrens Hosp Datteln, Dept Pediat Neurol, Datteln, Germany
[2] Med Univ Vienna, Dept Neurol, Div Neuropathol & Neurochem, Vienna, Austria
[3] Med Univ Vienna, Comprehens Ctr Pediat, Dept Pediat & Adolescent Med, Vienna, Austria
[4] Univ Witten Herdecke, Childrens Hosp Datteln, Dept Pediat Radiol, Datteln, Germany
[5] Med Univ Vienna, Dept Pediat & Adolescent Med, Div Pediat Pulmonol Allergol & Endocrinol, Vienna, Austria
[6] Olga Hosp, Dept Pediat, Div Pediat Neurol, Stuttgart, Germany
[7] Univ Augsburg, Augsburg Univ Hosp, Div Pediat Neurol, Clin Pediat, Augsburg, Germany
[8] Med Univ Innsbruck, Dept Pediat 1, Pediat Neurol, Innsbruck, Austria
[9] Childrens Hosp DRK Siegen, Dept Neuropediat, Siegen, Germany
[10] Univ Hosp Heidelberg, Ctr Child & Adolescent Med, Dept Gen Pediat, Div Neuropediat & Metab Med, Heidelberg, Germany
[11] Hosp Neuropediat & Neurol Rehabil, Schoen Klin Vogtareuth, Ctr Epilepsy Children & Adolescents, Vogtareuth, Germany
[12] Paracelsus Med Univ Salzburg, Res Inst Rehabil Transit & Palliat, Salzburg, Austria
[13] Tech Univ Munich, Sch Med, Kinderklin Munchen Schwabing, Dept Pediat,Pediat Neurol, Munich, Germany
[14] Tech Univ Munich, Dept Pediat, Social Pediat, Munich, Germany
[15] Hosp Dritter Orden, Dept Pediat & Adolescent Med, Munich, Germany
[16] Klinikum Mutterhaus Borromaerinnen, Dept Neuropediat, Trier, Germany
[17] Klinikum Leverkusen, Social Pediat Ctr, Dept Neuropediat, Leverkusen, Germany
[18] Klinikum Bayreuth, Dept Pediat, Bayreuth, Germany
[19] Barmherzige Bruder St Hedwig Hosp, Dept Neuropediat, Clin Pediat, Regensburg, Germany
[20] Univ Childrens Hosp Tubingen, Dept Pediat Neurol & Dev Med, Tubingen, Germany
[21] Childrens Hosp St Marien, Dept Pediat Neurol, Landshut, Germany
[22] Univ Med Ctr Schleswig Holstein, Dept Pediat, Kiel, Germany
[23] Bethanien Hosp, Dept Pediat, Moers, Germany
[24] Med Univ Innsbruck, Clin Dept Neurol, Innsbruck, Austria
[25] Med Univ Vienna, Dept Biomed Imaging & Image Guided Therapy, Vienna, Austria
[26] Klin Donaustadt, Dept Radiol, Vienna, Austria
[27] Univ Hosp Schleswig Holstein, Inst Clin Chem, Kiel, Germany
[28] Univ Hosp Schleswig Holstein, Dept Neurol, Kiel, Germany
[29] Med Univ Vienna, Comprehens Ctr Clin Neurosci & Mental Hlth, Vienna, Austria
基金
奥地利科学基金会;
关键词
Cerebellitis; MOG antibodies; Children; Autoimmune; MOG-ANTIBODIES; FEATURES; ASTROCYTOPATHY; AUTOIMMUNITY; DISORDERS; SPECTRUM;
D O I
10.1016/j.ejpn.2023.10.005
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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, GFAP alpha-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; GFAP alpha-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.
引用
收藏
页码:118 / 130
页数:13
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