Abstract Details

Title GFAP Astrocytopathy Presenting as Rhombo-meningoencephalitis with Initial Negative Antibody Testing

Topic Autoimmune Neurology

Presentation(s) P11 - Poster Session 11 (11:45 AM-12:45 PM)

Poster/Presentation
Number 1-004

Objective To describe a case of glial fibrillary acidic protein (GFAP) astrocytopathy with an initial presentation of rhombo-meningoencephalitis and negative cerebrospinal fluid (CSF) antibody testing, underscoring the diagnostic value of repeat testing.

Background GFAP astrocytopathy is a rare autoimmune syndrome most often presenting as meningoencephalomyelitis with CSF-specific GFAP-IgG. Patients typically develop subacute encephalopathy, longitudinal myelitis, or optic neuritis after a febrile prodrome. Brainstem and cerebellar features are recognized but usually occur alongside encephalopathy or myelitis. MRI may show radial perivascular enhancement, but diagnosis requires CSF antibody detection, as serum testing alone is insufficient.

Design/Methods A previously healthy 22-year-old male presented with fever and meningismus after Caribbean travel. Initial brain MRI was normal. One week later, he developed encephalopathy with brainstem and cerebellar dysfunction (vertical gaze palsy, nystagmus, hiccups, dysmetria) and ileus. MRI brain showed a faint right cerebellar T2 hyperintensity. Spine MRI revealed a longitudinally extensive cord lesion from T2–T8. CSF demonstrated lymphocytic pleocytosis and elevated protein. Infectious studies were negative. Autoimmune panel showed a low-titer anti-GQ1b, considered incidental. He received corticosteroids but soon deteriorated with status epilepticus and respiratory failure requiring intubation, after which plasmapheresis was added. Follow-up MRI demonstrated diffuse cortical sulcal T2 changes with leptomeningeal enhancement, and new thalamic, brainstem, and cerebellar lesions; the cord lesion persisted. Repeat CSF testing three weeks after the initial evaluation was positive for GFAP-IgG by IFA, confirmed by cell-based assay. Malignancy screening was negative.

Results NA

Conclusions This case illustrates GFAP astrocytopathy presenting with prominent rhombencephalitic features. It underscores three lessons: (1) GFAP astrocytopathy may mimic isolated brainstem or cerebellar syndromes; (2) early antibody testing may be negative, requiring repeat CSF evaluation; and (3) incidental low-titer antibodies must be interpreted with caution. GFAP astrocytopathy should be considered in patients with fever, encephalopathy, rhombencephalic dysfunction, and myelopathy when infectious studies are negative.

Authors/Disclosures
Sina Aghili-Mehrizi, MD PRESENTER	Dr. Aghili-Mehrizi has nothing to disclose.
Kyle A. Wuthrich, DO (USF Health Neurology)	Dr. Wuthrich has nothing to disclose.
Priyanka Atit, DO	Dr. Atit has nothing to disclose.
Haoming Pang, MD (University of South Florida, Tampa General Hospital)	Dr. Pang has nothing to disclose.
Ali Karimi	Mr. Karimi has nothing to disclose.
Avinash S. Kolli, MD	Dr. Kolli has nothing to disclose.
Rebecca L. Hurst, MD (USF College of Medicine)	Dr. Hurst has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for The Paul Wilkinson law firm.
Victoria Mazo, MD	Dr. Mazo has nothing to disclose.
Angelica Rivera Cruz, MD (University of South FLorida)	Dr. Rivera Cruz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Rivera Cruz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Jazz pharmaceutical. Dr. Rivera Cruz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for SK Life Science.

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