Table 1.
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. |