Abstract Details

Title A Case of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy(GFAP) Meningoencephalomyelitis Responsive to Infliximab

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-009

Objective

1. Describe a unique presentation and neuroimaging for autoimmune GFAP meningoencephalomyelitis.

2. Identify Infliximab as an effective steroid-sparing therapy.

Background A novel intracellular astrocyte IgG autoantibody against GFAP has recently been identified as a biomarker for an autoimmune meningoencephalomyelitis.

Design/Methods Case Report

Results A 25 year old African American man presents with 7 months of decline in mental state and generalized weakness. Prior to the onset of these symptoms he had an unintentional loss of 30kg due to persistent nausea, hiccups, and headache. He then developed generalized weakness along with gait instability, frequent falls, and tremor. He began to have audio-visual hallucinations and impairment of short-term memory. His symptoms have not improved on anti-psychotic medications.
He can only intermittently follow single-step commands and has sparse speech. He has intact cranial nerves, sensation, and reflexes. He has a high-frequency essential tremor and generalized weakness.
Neuroimaging reveals diffuse leptomeningeal thickening and enhancement to brain and spinal cord along with enhancement of nerve roots. CSF has elevated protein (233mg/dL), 5 oligoclonal bands, elevated ACE (6.3U/dL), and lymphocytic pleocytosis. CT chest is concerning for hilar lymphadenopathy.
With concern for neurosarcoidosis, he is started on high-dose steroids; however subsequent lymph node biopsy is negative for granulomatous disease. His mental state modestly improves on steroids with a rapid improvement following addition of infliximab. His CSF studies subsequently test positive for GFAP auto-antibodies.

Conclusions This case demonstrates autoimmune subacute meningoencephalitis due to astrocytopathy with GFAP auto-antibodies. Neuroimaging shows both leptomeningeal and nerve root enhancement, and his clinical condition significantly improves with the initiation of anti-TNF-alpha therapy with infliximab. The mechanism by which infliximab treats GFAP meningoencephalitis is unknown. However recent brain immunohistopathology demonstrated an extensive involvement of CD4+, CD3+, and CD8+ T cells, as well as B cells, plasma cells, and macrophages.

Authors/Disclosures
Justin Rodriguez, MD, PhD (UTHSCSA Dept of Neurology) PRESENTER	No disclosure on file
Rebecca Romero, MD, FAAN (UTHSCSA)	Dr. Romero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Romero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics . Dr. Romero has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Romero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon.

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