The present patients with positive GFAP-immunoglobulin G are highly similar to autoimmune GFAP astrocytopathy, described in a recent report. The features of the neuropathology and immunopathology of GFAP astrocytopathies were perivascular inflammation and loss of astrocytes and neurons.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune disease of the nervous system first defined in 2016. GFAP autoantibody, especially IgG that binds to GFAPα, has been reported in the cerebrospinal fluid (CSF) and serum of patients with GFAP astrocytopathy. The positive predictive value of GFAP antibody in the CSF is higher than in the serum. Tissue-based assay (TBA) and cell-based assay (CBA) are both recommended methods for the detection of GFAP antibody. GFAP astrocytopathy is accompanied by neoplasms, but the relationship between virus infection and GFAP astrocytopathy is unclear. GFAP antibody itself does not induce pathological changes; it is only a biomarker for the process of immune inflammation. The pathology of GFAP astrocytopathy in humans is heterogeneous. GFAP astrocytopathy is commonly diagnosed in individuals over 40 years old and most patients have an acute or subacute onset. Clinical manifestations include fever, headache, encephalopathy, involuntary movement, myelitis, and abnormal vision. Lesions involve the subcortical white matter, basal ganglia, hypothalamus, brainstem, cerebellum, and spinal cord. The characteristic MRI feature is brain linear perivascular radial gadolinium enhancement in the white matter perpendicular to the ventricle. Currently, there are no uniform diagnostic criteria or consensus for GFAP astrocytopathy and coexisting neural autoantibodies detected in the same patient make the diagnosis difficult. A standard treatment regimen is yet to be developed. Most GFAP astrocytopathy patients respond well to steroid therapy although some patients are prone to relapse or even die.

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Objective: The aim of this work was to report an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy with long-term follow-up in 7 patients. Methods: Antibodies were detected by indirect immunofluorescence assay and patient data were analyzed retrospectively. Results: Seven patients (4 female, 3 male) with ≥1 year follow-up were included. All patients had positive GFAP antibodies in their cerebral spinal fluid (CSF). Their median age at disease onset was 56 years (range 27-69) and the median disease duration was 1 year (range 1-4). In the initial attack, all patients received intravenous methylprednisolone (IVMP) followed by oral steroids, which were tapered. Six patients received intravenous immunoglobulin (IVIg). One patient had no response to IVIg and IVMP. Four patients received immunosuppressive agents. Five patients underwent the second lumbar puncture after treatment. CSF white blood cell counts, protein levels, and antibody titers were significantly decreased. CSF protein levels correlated positively with the Expanded Disability Status Scale score, which was elevated at each lumbar puncture. Four patients experienced relapse. To date, 6 patients had a bad prognosis, of which 2 died. Conclusions: Some patients with GFAP astrocytopathy experienced a poor response to treatment although they received steroids and immunosuppressive agents.