Pseudotumor cerebri complicating multisystem inflammatory syndrome in a child

Abstract

Purpose: To report a case of pseudotumor cerebri (PTC) in a child associated with multisystem inflammatory syndrome in children (MIS-C), associated with presumed coronavirus disease 2019. Methods: A previously healthy 11-year-old female child presented with a 4-day history of fever, headache, vomiting, and loose stools. Laboratory investigations revealed neutrophilic leukocytosis, and markers of inflammation (C-reactive protein, ferritin, and interleukin-6) were significantly elevated. Pharyngeal swab for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by polymerase chain reaction was negative while anti-SARS-CoV-2 antibody was highly reactive. Ophthalmic evaluation for transient visual obscurations during hospital course revealed swelling of the optic disc in both eyes. Spectral-domain optical coherence tomography and ultrasonography confirmed the ophthalmoscopic findings. There was no neurologic deficit. Magnetic resonance imaging of the brain and magnetic resonance venogram revealed no structural lesion. The opening pressure of cerebrospinal fluid (CSF) was 336 mm of water, and CSF composition was normal. Results: A diagnosis of PTC associated with MIS-C was made, and the child was treated with oral acetazolamide. Edema of the optic disc regressed following therapy, and the child is under follow-up. Conclusions: PTC can occur in association with MIS-C. Clinicians need to be aware of this potential neuro-ophthalmic complication in MIS-C. Prompt diagnosis and treatment can prevent visual loss.

A prospective long-term evaluation of the ocular findings of children followed with the diagnosis of multisystem inflammatory syndrome (long-term evaluation of ocular findings following MIS-C)

“…There are case reports of papilledema in MIS-C patients in the literature, albeit in a small number. Similar to the cases in our study, in the case report published by Divya et al, in the examination of an 11-year-old patient, the visual acuity was 20/ 20, and colour vision was complete in both eyes [9]. Similar to the one patient in our study, this patient was diagnosed with pseudotumor cerebri and received systemic acetazolamide therapy.…”

“…In the literature, the most frequently reported ocular finding in the acute phase in patients undergoing MIS-C is conjunctivitis, similar to Kawasaki disease, and less commonly punctuate epitheliopathy, anterior uveitis and papilledema [7][8][9][10][11][12]. Studies in the literature generally include the findings of MIS-C in the acute period.…”

Neurological manifestations of Kawasaki disease and multisystem inflammatory syndrome in children associated with COVID-19: A comparison of two different clinical entities

“…Verkuil et al and Divya et al observed two different cases of otherwise healthy adolescents affected by MIS-C who developed bilateral papilledema in the absence of any other neurological symptoms and of any lesions in MRI and magnetic resonance venography; increased pressure in cerebrospinal fluid was diagnostic for pseudotumor cerebri ( 106 , 107 ).…”

“…The pathophysiologic mechanisms at the basis of this relation are not clear but seem to be related to systemic inflammation: several inflammatory mediators (e.g., TNFa, IL-1, and IL-6) are known to be able to activate enzyme 11-beta-hydroxysteroid dehydrogenase that interacts in the homeostasis of intracranial pressure. Furthermore, encephalitis, cerebral venous thrombosis, coagulopathy, and hyperviscosity can impair cerebral spinal fluid absorption ( 106 ).…”

Cerebrospinal fluid (CSF) and idiopatic intracranial hypertension (IIH): a critical review

“…Other inflammatory diseases have also been associated with pseudotumor cerebri. 20,21 The contribution of inflammatory mechanisms and changes in cytokine levels have been proposed as one of the pathophysiological mechanisms of this disorder. One study found that one-third of IIH patients showed various patterns of oligoclonal bands (OCBs).…”