Unmasking of a Relapsing Encephalomyelitis After SARS-CoV-2 Infection and COVID-19 Vaccination

Abstract

Objective NA. Background Prior case studies suggest that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its vaccines may unmask neuroinflammatory conditions. We present a case of relapsing steroid-responsive encephalomyelitis after SARS-CoV-2 infection and subsequent COVID-19 vaccination. Design/Methods NA. Results A 47-year-old man with a history COVID-19 presented with subacute lower extremity weakness, erectile dysfunction, and gait instability with falls. His symptoms started several weeks after COVID-19 vaccination which he underwent 3 months after COVID-19 infection. His initial exam demonstrated weakness at the knees and ankles and extensor plantar responses. MRI demonstrated innumerable enhancing lesions involving the subcortical white matter, basal ganglia, thalami, brainstem, cerebellum, and the entire spinal cord parenchyma. CSF testing revealed a lymphocytic pleocytosis (10 WBC, 88% lymphocytes), and transient matched serum and CSF oligoclonal bands. Testing was unremarkable for infections, malignancies, primary demyelinating conditions, etc. He responded dramatically to five days of high dose methylprednisolone but had recurrence of symptoms with weaning of oral prednisone, requiring another pulse of IV steroids. After 2 months, his steroids were weaned again, with clinical and radiographic recurrence, requiring another course of IV steroids. He was subsequently transitioned to mycophenolate as a steroid-sparing agent. Literature review identified 20 additional cases of CNS neuroinflammatory disease after either SARS-CoV-2 infection or vaccination (11 transverse myelitis, 6 optic neuritis, 3 encephalomyelitis). Conclusions Our patient's steroid-dependency and relapsing course suggests unmasking of an underlying CNS neuroinflammatory condition. Temporal associations of neurological conditions with vaccinations or infections do not prove causality despite previous reports of such sequelae. Vaccines containing SARS-CoV-2 antigens may enhance autoimmunity by mechanisms including polyclonal activation, epitope spreading, or molecular mimicry. This case highlights that the resulting inflammation may be insidious and extensive, though treatable. As COVID-19 constitutes a life-threatening infection in some patients, the benefits of vaccination outweigh the smaller risk of unmasking an immune-related condition.