Abstract Details

Title An Atypical Case of anti-GFAP Astrocytopathy

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 055

Objective To describe a case of anti-GFAP astrocytopathy with atypical features.

Background Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune central nervous disorder that is typically preceded by viral illness (fevers, chills, malaise), followed by meningoencephalitis with possible features of psychosis, optic nerve edema without significant visual deficit, inflammatory CSF, mildly elevated intracranial pressure, and characteristic T1 post-gadolinium perivascular enhancement. We present an atypical case of GFAP astrocytopathy that broadens the clinical spectrum of the disease.

Design/Methods Case Report

Results A 31 year old male with a history of hypertension and obesity developed flu-like symptoms for one week. He then acutely developed blurry vision, for which he presented to the emergency department. He was treated for hypertensive urgency and subsequently discharged. However, his vision continued to deteriorate for which he presented to our hospital. Ophthalmologic exam revealed absence of light perception bilaterally, retinal hemorrhages and severe papilledema. MRI brain and orbits with contrast showed an empty sella and brain MRV was unremarkable. CSF revealed lymphocytic pleocytosis, elevated protein, oligoclonal bands and an opening pressure of 54. Shortly after, he developed encephalopathy with delusions and visual hallucinations. Three repeat lumbar punctures showed persistently elevated opening pressure and lymphocytic pleocytosis. Workup did not identify infection or occult malignancy. He was started on intravenous methylprednisolone and plasma exchange, with rapid resolution of his encephalopathy. Despite treatment with acetazolamide and bilateral optic nerve sheath fenestration, his vision did not improve. Subsequently, anti-GFAP antibody was detected in both serum and CSF, leading to diagnosis of anti-GFAP astrocytopathy. He was continued on a slow steroid taper and started on monthly pulse cyclophosphamide, given severity of vision impairment.

Conclusions Here, we present previously undescribed manifestations of anti-GFAP astrocytopathy including rapid and profound visual loss associated with severe intracranial hypertension, thereby expanding the clinical spectrum of this rare disorder.

Authors/Disclosures
Maxime Jean, MD, PhD (University of Rochester Medical Center) PRESENTER	Dr. Jean has nothing to disclose.
David Sandness, MD (University of Rochester Medical Center)	Dr. Sandness has nothing to disclose.
Ryan Canissario, MD (Virginia Commonwealth University Health System)	Dr. Canissario has nothing to disclose.
Lawrence M. Samkoff, MD, FAAN (University of Rochester School of Medicine and Dentistry)	Dr. Samkoff has nothing to disclose.

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