Pseudotumor cerebri syndrome associated with MIS-C: a case report

Abstract

A previously healthy 14-year-old girl (body-mass index 19·2 kg/m2) presented to our hospital (Children's Hospital of Philadelphia, Philadelphia, PA, USA) with 5 days of fever, headache, rash, diarrhoea, and dyspnoea. 2 months previously, she had presented to our emergency department with cough, headache, and myalgias of unknown cause; however, she was discharged without hospital admission. Initial laboratory assessment upon current presentation showed leukopenia, increased C-reactive protein (34·3 mg/dL) and fibrinogen (657 mg/dL), normal international normalised ratio and partial thromboplastin time. Respiratory failure and septic shock required mechanical ventilation and vasopressors. Chest CT showed diffuse ground glass airspace opacities with subpleural sparing. She was given empiric broad spectrum antibiotics, including 9 days of doxycycline, and hydrocortisone for shock. Nasopharyngeal and deep endotracheal sampling for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by real-time PCR were negative. Echocardiogram detected diffuse dilation of the right coronary artery. Presumed atypical Kawasaki shock syndrome was treated with intravenous immunoglobulin and intravenous methylprednisolone. She was extubated after 6 days of ventilation, and then she manifested a new esotropia. Visual acuity, colour vision, and pupils were normal. No conjunctival injection or anterior segment inflammation was present. A right-eye abduction deficit, consistent with an abducens palsy, was present. Dilated fundus examination revealed bilateral papilloedema with left-disc haemorrhages. Neurological examination was otherwise normal. MRI of the brain and magnetic resonance venogram revealed abnormalities consistent with increased intracranial pressure (appendix pp 1–3). Lumbar puncture on day 11 after admission to hospital revealed an opening pressure of 36 cm H20, two white blood cells per μL, and normal glucose and protein. Acetazolamide 250 mg twice daily and an oral prednisone taper were prescribed. After 14 days in hospital, the patient was discharged. After discharge, IgG qualitative testing returned positive, suggestive of SARS-CoV-2 infection. 2-month follow-up revealed resolution of papilloedema, disc haemorrhages, and abducens palsy. She reported non-compliance with the prescribed acetazolamide. Multisystem inflammatory syndrome in children (MIS-C) is a recently reported paediatric syndrome associated with SARS-CoV-2 infection.1CDC Health Alert NetworkMultisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19). US Centers for Disease Control and Prevention.https://emergency.cdc.gov/han/2020/han00432.aspDate: May 14, 2020Date accessed: May 15, 2020Google Scholar Based on our patient's papilloedema, abducens palsy, normal brain parenchyma, and increased cerebrospinal fluid opening pressure with normal constituents, in the setting of MIS-C, she meets criteria for secondary pseudotumor cerebri syndrome (PTCS).2Friedman DI Liu GT Digre KB Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children.Neurology. 2013; 81: 1159-1165Crossref PubMed Scopus (689) Google Scholar, 3Paley GL Sheldon CA Burrows EK Chilutti MR Liu GT McCormack SE Overweight and obesity in pediatric secondary pseudotumor cerebri syndrome.Am J Ophthalmol. 2015; 159: 344-352Summary Full Text Full Text PDF PubMed Scopus (29) Google Scholar PTCS might be primary (idiopathic intracranial hypertension); however, inflammatory and infectious conditions have been implicated as secondary causes.4Rangwala LM Liu GT Pediatric idiopathic intracranial hypertension.Surv Ophthalmol. 2007; 52: 597-617Summary Full Text Full Text PDF PubMed Scopus (203) Google Scholar Although cerebrospinal fluid dynamics were altered in our patient with MIS-C, the mechanism remains unclear. Doxycycline was thought to be non-contributory because doxycycline-related PTCS typically occurs after 1–2 months of use.3Paley GL Sheldon CA Burrows EK Chilutti MR Liu GT McCormack SE Overweight and obesity in pediatric secondary pseudotumor cerebri syndrome.Am J Ophthalmol. 2015; 159: 344-352Summary Full Text Full Text PDF PubMed Scopus (29) Google Scholar As our understanding of MIS-C evolves, an ocular fundus examination might be required as part of a multi-system approach to assess patients with suspected MIS-C. PTCS is a potentially vision-threatening condition and should be considered in this clinical setting. We declare no competing interests. Download .pdf (.36 MB) Help with pdf files Supplementary appendix