Abstract Details

Title Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy Masquerading as Varicella-zoster Virus (VZV) Meningoencephalomyelitis: A Case Report

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (5:00 PM-6:00 PM)

Poster/Presentation
Number 1-012

Objective To describe a case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, highlighting the importance of considering this entity in the differential diagnosis of presumed central nervous system infections.

Background GFAP astrocytopathy is a rare autoimmune neuroinflammatory disorder with a broad spectrum of clinical manifestations including meningitis, encephalitis, and/or myelitis. Its presentation can closely mimic infectious meningoencephalomyelitis, particularly when meningitis is the presenting feature.

Design/Methods N/A

Results A 24-year-old man with no significant medical history presented with several days of fever, headache, meningismus, and photophobia. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis (320 cells/µL), elevated protein (186 mg/dL), and low glucose (35 mg/dL). Varicella-zoster virus (VZV) DNA was detected by a multiplex meningitis/encephalitis PCR panel. Despite prompt initiation of intravenous acyclovir, he developed progressive encephalopathy, seizures, respiratory failure, and quadriparesis. MRI of the brain and spine revealed multiple linear T2-hyperintense lesions in the periventricular white matter, a cytotoxic lesion of the corpus callosum (CLOCC), and a longitudinally extensive spinal cord lesion spanning the upper cervical to lower thoracic segments. High-dose intravenous corticosteroids were initiated, followed by plasma exchange and intravenous immunoglobulin, resulting in gradual neurologic improvement. CSF GFAP-IgG later returned positive at high titer (1:64) by cell-based assay, confirming a diagnosis of autoimmune GFAP astrocytopathy. The patient was transitioned to a prolonged oral corticosteroid taper over several months, with continued functional recovery. A stand-alone (non-multiplex) CSF VZV PCR later returned negative, suggesting a false-positive result on the initial multiplex assay.

Conclusions Autoimmune GFAP astrocytopathy can closely mimic viral meningoencephalomyelitis, and false-positive multiplex PCR results may confound the diagnosis. This entity should be considered in patients with presumed infectious meningitis and/or encephalomyelitis unresponsive to antiviral therapy, as early recognition and immunotherapy can lead to substantial recovery.

Authors/Disclosures
Maria L. Rodriguez, MD PRESENTER	Ms. Rodriguez has nothing to disclose.
Jessa Alcaide, MD	Dr. Alcaide has nothing to disclose.
Yaswanth Chintaluru, MD	Yaswanth Chintaluru, MD has stock in Gravity Medical Technology.
Athena Dong, MD	Dr. Dong has nothing to disclose.
Cynthia Janku, MD	Dr. Janku has nothing to disclose.
Andrew Ovakimyan, MD	Dr. Ovakimyan has nothing to disclose.
Rumyar V. Ardakani, MD	Dr. Ardakani has nothing to disclose.

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