Black holes and high levels of neurofilaments in glial fibrillary acidic protein – astrocytopathy: a case report
Fecha
2020Language
en
Materia
Resumen
Background and purpose: Glial fibrillary acidic protein (GFAP) is an intracellular protein of the astrocytic cytoskeleton. Recently, autoantibodies to GFAP detected by cell-based assay in cerebrospinal fluid (CSF) or serum have been implicated in cerebral astrocytopathy, presenting predominantly with autoimmune meningoencephalomyelitis. However, the phenotypic spectrum, prognosis and therapeutics of this new entity remain to be elucidated. Methods: Herein, we report radiological, CSF and serological findings during disease exacerbation and remission, from a patient with autoimmune GFAP astrocytopathy, presenting as an immunotherapy responsive GFAP IgG-associated meningoencephalomyelitis. Results: Brain and spine magnetic resonance imaging revealed meningeal enhancement, T2 hyperintensities, black holes, significant sulci widening and spinal atrophy. In addition, high levels of neurofilaments (NfL) and GFAP were also identified during disease exacerbation, consistent with the appearance of the black holes. Conclusions: To date, black holes and atrophy have never been reported before in autoimmune GFAP astrocytopathy. These findings, combined with the high levels of GFAP and NfL, suggest the existence of an underlying neurodegenerative mechanism in addition to the known inflammatory response. Further studies are needed to elucidate the pathomechanism of GFAP-astrocytopathies. © 2020 European Academy of Neurology
Colecciones
Ítems relacionados
Mostrando ítems relacionados por Título, autor o materia.
-
The expression of the epitope H recognized by the monoclonal antibody H is higher in astrocytomas compared to anaplastic astrocytomas and glioblastomas
Arvanitis, D. L.; Arvanitis, L. D.; Panourias, I. G.; Kitsoulis, P.; Kanavaros, P. (2005)The epitope H contains an O-linked N-acetylglucosamine residue in a specific conformation and/or environment recognized by the monoclonal antibody H (mAbH). mAbH stains two bands with Mr x10-3 of 209 and 62 in lysates of ... -
Neural Differentiation of Human Dental Mesenchymal Stem Cells Induced by ATRA and UDP-4: A Comparative Study
Koutsoumparis A.E., Patsiarika A., Tsingotjidou A., Pappas I., Tsiftsoglou A.S. (2022)Human mesenchymal stem cells (MSC) are multipotent stem cells, which are isolated from various sources. Currently, there is a worldwide interest for dental MSC to be used against neurodegenerative diseases, since they ... -
Humoral response in experimental autoimmune encephalomyelitis targets neural precursor cells in the central nervous system of naive rodents
Kesidou E., Touloumi O., Lagoudaki R., Nousiopoulou E., Theotokis P., Poulatsidou K.-N., Boziki M., Kofidou E., Delivanoglou N., Minti F., Hadjigeorgiou G., Grigoriadis N., Simeonidou C. (2017)Background: Neural precursor cells (NPCs) located in the subventricular zone (SVZ), a well-defined NPC niche, play a crucial role in central nervous system (CNS) homeostasis. Moreover, NPCs are involved in the endogenous ...