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

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.

Enteroviral Rhombencephalitis with Abducens Nerve Palsy and Cardio-Pulmonary Failure in a 2-Year-Old Boy

Enterovirus infection is endemic in many areas, especially in Southeast Asia. Enterovirus infection with severe complications (EVSC) is life-threatening, and timely diagnosis and management are crucial for successful management. Here, we report on a 2-year-old boy with hand, foot, and mouth disease. Myoclonic jerks developed and left abducens nerve palsy followed. Brain magnetic resonance imaging (MRI) showed rhombencephalitis. Pulmonary edema and cardiopulmonary failure developed, and intravenous immunoglobulin and extracorporeal membrane oxygenation were administered. He had a tracheostomy with home ventilator use after 64 days of hospitalization. At a 5-year follow-up, his neurodevelopment was normal with complete recovery from the abducens nerve palsy. The progress of EVSC may be rapid and fulminant, and timely diagnosis is critical for patient prognosis and outcomes. The presence of abducens nerve palsy is an indicator of enteroviral rhombencephalitis, and immediate and appropriate management is suggested.

Post-infectious rhombencephalitis after coronavirus-19 infection: A case report and literature review.

A wide number of neurological manifestations have been described in association with coronavirus disease 19 (COVID-19). We describe an unusual case of a young man who developed severe rhombencephalitis after COVID-19. He demonstrated clinical and radiological improvement with high dose corticosteroids, plasma exchange and intravenous immune globulin. Our findings, along with previously reported cases that we review here, support an autoimmune para- or post-infectious mechanism and highlight a possible role for immunotherapy in patients with rhombencephalitis after COVID-19.

Lessons of the month 1: A case of rhombencephalitis as a rare complication of acute COVID-19 infection.

A 40-year-old man developed acute brainstem dysfunction 3 days after hospital admission with symptoms of the novel SARS-CoV-2 infection (COVID-19). Magnetic resonance imaging showed changes in keeping with inflammation of the brainstem and the upper cervical cord, leading to a diagnosis of rhombencephalitis. No other cause explained the patient's abnormal neurological findings. He was managed conservatively with rapid spontaneous improvement in some of his neurological signs and was discharged home with continued neurology follow up.