Skip to main content
. 2018 Dec 5;9:2802. doi: 10.3389/fimmu.2018.02802

Table 1.

Literatures of human GFAP astocytopathy.

Author Year Country n Pathological examination Main findings
Fang et al. (2) 2016 USA 16 No The first paper to described human GFAP astrocytopathy. They describe GFAP-IgG found in serum or cerebrospinal fluid that is specific for a cytosolic intermediate filament protein of astrocytes.
Flanagan et al. (3) 2017 USA 102 Yes Retrospectively analyzed 102 GFAP-IgG positive patients. Specificity of serum and CSF testing, the clinical and radiological phenotype evaluate, the significance of coexisting antibodies, and therapy responses were reported. CSF-GFAPαa-IgG is highly specific for an immunotherapy-responsive autoimmune CNS disorder.
Yang et al. (4) 2017 China 7 Yes To assess the treatment response in seven GFAP-IgG-positive patients with long-term follow-up. Some patients with GFAP astrocytopathy experienced a poor response to treatment although they received steroids and immunosuppressive agents,
Long et al. (5) 2018 China 19 Yes To describe the clinical, radiological and pathological features in 19 patients with CSF-GFAP-IgG in CSF. The features of the neuropathology and immunopathology of GFAP astrocytopathies were perivascular inflammation and loss of astrocytes and neurons.
Yang et al. (6) 2018 China 10 Yes To study overlapping syndromes in autoimmune GFAP astrocytopathy. Overlapping antibodies are common in GFAP astrocytopathy.
Iorio et al. (7) 2018 Italy 22 Yes To report the clinical and immunological characteristics of 22 new patients with GFAP-IgG. GFAP autoimmunity is not rare. The clinical spectrum encompasses meningoencephalitis, myelitis, movement disorders, epilepsy and cerebellar ataxia. coexisting neurological and systemic autoimmunity are relatively common. Immunotherapy is beneficial in most cases.
Zarkali et al. (8) 2018 UK 1 No To report a young man presenting with subacute meningoencephalitis and subsequent myelitis, and discuss the typical presentation and management of this severe but treatable condition.
Shu et al. (9) 2018 China 1 Yes To examined brain biopsy sections from a patient with autoimmune GFAP astrocytopathy using hematoxylin and eosin and Luxol fast blue staining, and immunostaining with antibodies.
Martin et al. (10) 2018 USA 1 Yes To report a 13-years-old girl with acute-onset meningoencephalitis and incidental finding of ovarian teratoma was found to have coexisting anti-NMDA-R and GFAP antibodies present in her cerebrospinal fluid.
Li et al. (11) 2018 China 1 No To report a case of autoimmune GFAP astrocytopathy after herpes simplex viral encephalitis.
Dubey et al. (13)Sechi et al. (18) 20182018 USAUSA 90 13 YesNo This study demonstrates CSF GFAPα-IgG is a specific autoimmune meningoencephalomyelitis biomarker, with favorable corticosteroid response. Lack of response should prompt evaluation for co-existing NMDA-R-IgG or malignancy.
This study found that spinal cord lesions in GFAP-IgG myelitis were commonly longitudinally extensive (≥80%) and centrally located. Compare to AQP4-IgG lesions, they were more subtle lesions with poorly defined margins and less swelling. In GFAP-IgG myelitis, spinal cord central canal, punctate or leptomeningeal enhancement was typical.