GFAP astrocytopathy presenting after COVID-19 infection with meningoencephalomyelitis and good recovery with immunotherapy (P14-4.001)

Abstract

<h3>Objective:</h3> To describe a case of post-COVID-19 GFAP astrocytopathy presenting with meningoencephalomyelitis. <h3>Background:</h3> Glial fibrillary acidic protein (GFAP) astrocytopathy is a newly described autoimmune disorder that presents with meningoencephalitis, myelitis and cerebellar dysfunction. Differential diagnosis includes inflammatory, neoplastic and infectious etiologies. GFAP astrocytopathy associated meningoencephalomyelitis is a rare, but treatable neurologic sequela of COVID-19. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 62-year-old man presented with progressive confusion, recurrent falls, and weight loss with initial symptom onset during COVID-19 infection 6 weeks prior. MRI showed patchy parenchymal and ependymal enhancement in the brainstem and temporal lobe as well as T2 hyperintensity throughout the cervical and thoracic cord consistent with transverse myelitis. Four lumbar punctures demonstrated elevated protein between 100–200 mg/dl and significant lymphocytic pleocytosis with 300–600 nucleated cells. Extensive infectious work-up was negative. Malignancy work-up showed PET-CT avidity in the spinal cord and positive MYD88 mutation in the CSF, possibly indicating a lymphoproliferative condition. However, CSF cytology, CSF flow cytometry and a bone marrow biopsy showed no evidence of malignancy on repeated testing. CSF autoimmune encephalopathy panel demonstrated high titers of GFAP antibodies. Initial treatment included pulse-dose steroids and IVIG with minimal improvement in cognition. He was then treated with pulse-dose steroids and rituximab with markedly improved cognition, motor function and appetite. Subsequent 2-month MRI demonstrated partial resolution of his cerebral and spinal lesions. <h3>Conclusions:</h3> Subacute meningoencephalitis, with or without myelitis, is a typical presentation of GFAP astrocytopathy. Other symptoms include ataxia, blurred vision with optic disc edema, dysautonomia, seizures, area postrema syndrome and psychiatric symptoms. Most patients have a steroid responsive monophasic course, but approximately 20% of patients have relapsing disease. GFAP astrocytopathy should be on the differential for meningoencephalomyelitis, especially with infectious prodrome, such as COVID-19 infection. Early recognition and treatment can lead to improvement of neurologic deficits and a good outcome. <b>Disclosure:</b> Dr. Song has nothing to disclose. Dr. Oetjen has nothing to disclose. Dr. Harrold has nothing to disclose. Dr. Dietrich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Dietrich has received publishing royalties from a publication relating to health care. Kien-Ninh Ly has nothing to disclose. Dr. Venna has nothing to disclose.