High-dose corticosteroid therapy was associated with marked clinical and neuroimaging improvement in a patient with autoimmune glial fibrillary acidic protein astrocytopathy, according to a single-patient case report.
A 19-year-old woman presented with subacute meningoencephalitic symptoms, including intermittent fever, pulsatile temporal headache, transient loss of consciousness, and blurred vision. Magnetic resonance imaging (MRI) revealed multifocal T1/T2 hyperintensities with perivascular enhancement in the periventricular regions and pons. Additional imaging findings described in the report’s figures included involvement of the corona radiata and cervical spine.
Cerebrospinal fluid analysis showed lymphocytic pleocytosis with markedly elevated protein and pressure.
Following high-dose corticosteroid pulse therapy, the patient experienced marked improvement in both clinical manifestations and neuroimaging findings.
The report, published in Annals of Clinical and Translational Neurology, describes an interactive case (interACTN Case #56) and does not include comparator groups or quantitative effect estimates. The findings are descriptive and limited to a single clinical observation. No long-term outcomes or recurrence data were reported.
Autoimmune glial fibrillary acidic protein astrocytopathy is a rare inflammatory central nervous system disorder that should be considered in patients with subacute meningoencephalitic symptoms and characteristic MRI features.
Diagnosis relies on detection of glial fibrillary acidic protein antibodies in the cerebrospinal fluid in combination with clinical and radiologic findings.
High-dose corticosteroids, often used with intravenous immunoglobulin, are considered first-line therapy and are typically associated with clinical improvement.
Symmetrical radial periventricular enhancement on MRI is a hallmark feature of the condition and may aid in diagnosis. Because the disorder can be associated with underlying malignancies, tumor screening is recommended following diagnosis.
The researchers reported no conflicts of interest.
