2021 CMSC Annual Meeting

Immunotherapy-Responsive Post COVID-19 Central Nervous System Inflammatory Syndrome, a Case Report


Background: COVID 19 virus can infect the central and peripheral nervous system with or without other systemic manifestations of infection. SARS-CoV-2 interacts with the ACE-2 receptor, which is expressed in the central nervous system predominantly in the thalamic nuclei, cerebellum, and inferior olivary nuclei facilitating viral attachment, multiplication, and damage to neuronal tissue. Headaches are the most common neurological symptom and serious neurological disease is rare. Objectives: To report a case of COVID-19 infection in a 44-year-old woman leading to an inflammatory central nervous system (CNS) syndrome with convulsive and nonconvulsive status epilepticus. Methods: Case report. Results: 44-year-old, unknown-handed woman with past medical history of depression and chronic anemia due to menorrhagia from large uterine fibroids was initially admitted due to hypovolemic shock caused by menorrhagia, was also found to have COVID-19 infection. She returned 3 weeks later and was admitted to our intensive care unit due focal motor impaired awareness seizures and non-convulsive status epilepticus requiring intubation for airway protection. Magnetic resonance imaging (MRI) of the brain with and without contrast showed multifocal areas of abnormal T2 FLAIR hyperintensities in supratentorial and infratentorial gray and white matter, initially more pronounced in the left hemisphere with no contrast enhancement. Cerebrospinal fluid (CSF) studies revealed white blood cells (WBC) 5, glucose 55, protein 69, IGG index elevated at 10.1 with an otherwise unremarkable infectious and autoimmune workup. PCR for SARS COV-2 in CSF was not tested. Computed tomography of the chest, abdomen and pelvis was negative for malignancy and CSF autoimmune paraneoplastic panel was negative. Repeat MRI brain 8 days later demonstrated worsening of cortical and subcortical abnormalities in the right cerebral hemisphere. EEG showed bilateral independent lateralized periodic discharges with evolution, consistent with nonconvulsive status epilepticus. She was treated with methylprednisolone 1000 mg for 5 days without improvement followed by 5 doses of intravenous immunoglobulin therapy and ultimately required 10 day course of plasmapheresis. Her seizures were refractory to 3 antiseizure medications (levetiracetam 2000 mg BID, lacosamide 150 mg BID, oxcarbazepine 600 mg BID). After completion of immunotherapy, her clinical and electrographic seizures and her MRI findings resolved, though she had residual severe neuropsychiatric dysfunction. Conclusions: Although rare, inflammatory CNS syndromes are possible after COVID-19 infection. Immune-mediated injury occurs due to increased levels of inflammatory cytokines and activation of macrophages, T lymphocytes and endothelial cells. We hypothesize that these patients might improve with immunotherapy.