Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: A Case Series of Pediatric and Adult Patients.
Christopher Phillips1, Frederick Bassal2
1UC Davis Neurology Residency, 2University of California Davis Health Child Neurology
Objective:
Characterize the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy through a case series to aid in early recognition and management of this condition.
Background:
Autoimmune GFAP astrocytopathy is a recently identified and uncommon inflammatory central nervous system disorder with clinical phenotypic variability.
Design/Methods:
4 sequential cases of autoimmune GFAP astrocytopathy occurring at University of California, Davis between 2022 - 2024 were retrospectively reviewed to analyze the presenting syndromes, diagnostic testing results, treatment courses, and clinical outcomes.
Results:

The ages ranged from 12 to 24. Among these cases, there were two females, both adults. The other two were pediatric males. Presenting clinical syndromes varied significantly, including acute and subacute encephalopathy, subacute progressive psychosis, and subacute meningismus without encephalopathy. 3 cases also had fever, abdominal pain, nausea and emesis at onset of neurologic symptoms. No cases had a clear prodromal illness. CSF studies showed lymphocytic pleocytosis in 3 cases and protein elevation in all cases. Titers of anti-GFAP IgG in CSF ranged from 1:8 to 1:64. MRI of brain and spine showed stigmata of elevated ICP in 2 cases, leptomeningeal enhancement in 1 case, parenchymal enhancement in 1 case, and was normal in one case. EEG revealed subclinical seizures in 1 case and was normal in others. All cases were treated with immunotherapy, including intravenous methylprednisolone in all cases, plasmapheresis in 3 cases, and intravenous immunoglobulin in 1 case. 2 cases had worsening of symptoms requiring additional treatment. Maintenance immunotherapy with rituximab was started in 3 cases, one of which also received cyclophosphamide. 2 of 4 cases had significant symptomatic improvement with treatment, with the pediatric cases demonstrating minimal improvement. There were no mortalities.

Conclusions:
In this case series of sequential pediatric and adult cases of autoimmune GFAP astrocytopathy, there was significant variability in clinical phenotype and outcomes, despite timely initiation of immunotherapy.
10.1212/WNL.0000000000208420
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