Sommer, Simon and Panzer, Andreas and Bertolini, Annikki and Cleaveland, Robert and Jain, Vivek and Kapanci, Tugba and Derichs, Ute and Geis, Tobias and Neu, Axel and Loehr-Nilles, Christa and Aeschimann-Huhn, Rahel and Flotats-Bastardas, Marina and Deiva, Kumaran and Armangue, Thais and Olive-Cirera, Gemma and Kannoth, Sudheeran and Koy, Anne and Meirson, Hadas and Fattal-Valevski, Aviva and Ganelin-Cohen, Esther and Losch, Heike and Horne, Annacarin and Wickstroem, Ronny and Dargvainiene, Justina and Leypoldt, Frank and Rostasy, Kevin (2025) Spectrum of Clinical and Imaging Features of Children With GFAP Astrocytopathy. NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION, 12 (1): e200327. ISSN 2332-7812,
Full text not available from this repository. (Request a copy)Abstract
Background and ObjectivesGlial fibrillary acidic protein (GFAP) antibodies (abs) have been described primarily in adults with a spectrum of autoimmune-mediated diseases. In children, data on clinical and neuroradiologic features of children with autoimmune GFAP astrocytopathy are limited. The aim of this study was to describe the clinical and radiologic features in children with GFAP-ab-associated diseases.MethodsWe retrospectively recruited children from 13 clinical centers between 2020 and 2023 who (1) tested positive for GFAP-ab in serum and/or CSF and (2) of whom a complete clinical and MRI data set was available.ResultsWe identified and included 15 children (5 girls, 10 boys). The median age at onset was 9.9 years (range: 2-16 years). All children presented with features of AE or meningitis, acute cerebellitis, or transverse myelitis. CSF pleocytosis was common (13/15, median 245 cells/mu L), and 13 (87%) of 15 harbored GFAP-abs in their CSF, 8 (53%) of whom did not have detectable GFAP-abs in their serum. MRI was abnormal in 15 (100%) of 15 children: Specific patterns included confluent lesions in the pons or caudate nucleus (11/15; 73%), peri-aqueductal regions (13/15; 87%), and spinal cord (6/10; 60%). 12 children had a favorable outcome (mRS score of </= 1). Two patients died in the acute phase or during follow-up.DiscussionGFAP-ab-associated diseases encompass a wide spectrum of clinical presentation associated with a particular set of MRI features clearly distinct to other antibody-mediated diseases or MOGAD. We recommend that testing for GFAP-abs in serum and CSF be included in the workup of children with AE, particularly if brainstem involvement occurs.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | AUTOIMMUNE; ANTIBODIES; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Kinder- und Jugendmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 31 Mar 2026 07:11 |
| Last Modified: | 31 Mar 2026 07:11 |
| URI: | https://pred.uni-regensburg.de/id/eprint/67875 |
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