TY - JOUR
TI - Black holes and high levels of neurofilaments in glial fibrillary acidic protein – astrocytopathy: a case report
AU - Papa, A.
AU - Tzartos, J.S.
AU - Sakoutis, G.
AU - Dardiotis, E.
AU - Alexiou, E.
AU - Breza, M.
AU - Velonakis, G.
AU - Papamichalis, P.
AU - Mpampalis, D.
AU - Komnos, A.
AU - Karagiorgou, A.
AU - Papakonstantinou, A.
AU - Kilidireas, C.
AU - Hadjigeorgiou, G.M.
JO - European Journal of Paediatric Neurology
PY - 2020
VL - 27
TODO - 11
SP - 2381-2384
PB - Wiley-Blackwell Publishing Ltd
SN - 1090-3798
TODO - 10.1111/ene.14244
TODO - gadolinium;  glial fibrillary acidic protein;  glucose;  immunoglobulin G;  methylprednisolone;  mycophenolate mofetil;  prednisolone;  protein;  protein antibody;  autoantibody;  glial fibrillary acidic protein, adult;  analysis;  antibody titer;  Article;  autoimmune disease;  autoimmune glial fibrillary acidic astrocytopathy;  autoimmune glial fibrillary acidic astrocytopathy;  capsula interna;  case report;  cerebellum;  cerebrospinal fluid culture;  clinical article;  consciousness disorder;  contrast enhancement;  deterioration;  diffusion weighted imaging;  disease exacerbation;  drug dose reduction;  encephalomyelitis;  enzyme linked immunosorbent assay;  fever;  Glasgow coma scale;  headache;  hemisphere;  human;  intensive care unit;  laboratory;  leg muscle;  leukocyte count;  limb weakness;  lumbar puncture;  male;  meningoencephalitis;  neurofilament;  neuroimaging;  neurologic disease;  nuclear magnetic resonance imaging;  plasmapheresis;  priority journal;  protein cerebrospinal fluid level;  remission;  spinal cord atrophy;  astrocyte;  intermediate filament;  neurologic disease, Astrocytes;  Autoantibodies;  Autoimmune Diseases of the Nervous System;  Glial Fibrillary Acidic Protein;  Humans;  Intermediate Filaments
TODO - 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
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