Refractory Rhombencephalitis Secondary to SARS-CoV-2 Infection: A Case Report

ABSTRACT

Objective: To describe a case of rhombencephalitis secondary to severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) Background: Rhombencephalitis is an inflammation of the brainstem and cerebellum. Etiologies include infectious, inflammatory, and autoimmune causes. Rhombencephalitis has varied presentations but frequently includes encephalopathy, cranial neuropathies, long tract signs and cerebellar dysfunction. To date, SARS-CoV-2 has been reported as the cause of rhombencephalitis in 4 cases. Design/Methods: Authors searched PubMed and Google Scholar for articles using the keywords “COVID-19”, SARS-CoV-2', “Rhomboencephalitis”, “Rhombencephalitis”. Results: 30-year-old African American man with poorly controlled type 1 diabetes mellitus presented with dysgeusia, slurred speech, night sweats, left-sided hypoesthesia, paresthesias, ataxic gait, and light-headedness. Exam was notable for, left-sided hypoesthesia of the face and left upper extremity weakness as well as ataxia. MRI brain revealed diffuse pontine edema and central areas of diffusion restriction. COVID-19 nasal PCR and COVID-19 IgG antibodies were positive. Extensive infectious, autoimmune and paraneoplastic workup was unrevealing. Pulse-dose steroids resulted in improvement of edema and patient was discharged with diagnosis of a monophasic infectious rhombencephalitis due to COVID-19. Patient re-presented 8 days following discharge with acute left-sided headache and vomiting. Exam was notable for mild cranial nerve seven palsy and ataxia in all extremities. MRI brain displayed increased edema, mass effect and enhancement throughout the brainstem extending superiorly to include optic tracts and hypothalamus. CSF studies were remarkable for leukocytosis and increased protein. Repeat infectious, autoimmune and paraneoplastic studies again negative. Re-treatment with pulse-dose steroids followed by prolonged taper resulted in clinical and radiographic improvement at 1 month follow-up. Conclusions: The complete picture of neurological sequelae from COVID-19 is developing as the pandemic continues. Our case adds to the literature of SARS-CoV-2 associated rhombencephalitis and highlights the need for close monitoring and slow titration of immunotherapies such as steroids to minimize the potentially devasting effects of rhombencephalitis.