Cortical unihemispheric brain edema (CUBE) due to a multi‐system inflammatory syndrome in adults (MIS‐A)

Abstract

Unilateral hemispheric edema may occur with or without seizures. It is a rare entity, found more often in children, than in adults. As many of these patients develop pronounced unilateral complete cortical hemispheric (uni-hemispheric) edema on MRI, we have used the term "cortical unihemispheric brain edema" (CUBE) to describe this entity. We describe a case of CUBE occurring along with a post COVID multi-system inflammatory syndrome in adults (MIS-A). A 30-yearold man was admitted with status epilepticus. He was found to have CUBE and features of MIS-A. He had a history of mild COVID-19 illness one month earlier.He was noted to have persistently elevated inflammatory markers and multiorgan dysfunction compatible with MIS-A. We review the cases of CUBE in the literature and describe the features and etiology of this uncommon syndrome. Furthermore, we discuss the differences between two types of CUBE; cytotoxic CUBE (CUBE-C) and vasogenic CUBE (CUBE-V).

Multisystem inflammatory syndrome in adults: Characteristics, treatment, and outcomes

“…Uncommonly, 9 (5%) of 195 patients had arterial or venous thrombosis, and 1 had severe mononeuritis multiplex, affecting the right median and facial nerves and both ulnar, tibial, peroneal, and sural nerves 16 . Finally, systemic capillary leak syndrome, spontaneous coronary artery dissection, severe inflammatory myopathy, cortical unihemispheric brain edema, thyroiditis, refractory status epilepticus, ischemic stroke, and subconjunctival hemorrhage have been rarely reported as clinical manifestations of MIS‐A 28,30,31,61–63 …”

“…F I G U R E 1 Geographical distribution of reported cases of multisystem inflammatory syndrome in adults (as of October 26, 2022) 16,[27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43] The first reported MIS-A case was a 45-year-old male who developed fever for more than 5 days, erythema multiforme-like rash, bilateral nonexudative conjunctivitis, erythema or cracking of the lips, and unilateral cervical lymphadenopathy after caring for his wife with SARS-CoV-2 infection 2 weeks earlier. 25 He had no significant past medical history and denied respiratory symptoms on presentation.…”

“…16 Finally, systemic capillary leak syndrome, spontaneous coronary artery dissection, severe inflammatory myopathy, cortical unihemispheric brain edema, thyroiditis, refractory status epilepticus, ischemic stroke, and subconjunctival hemorrhage have been rarely reported as clinical manifestations of MIS-A. 28,30,31,[61][62][63] Overall, the clinical features of MIS-A, including fever, gastrointestinal, and cardiovascular involvement, are similar to those of MIS-C. 16,19,53 These findings were consistent with the comparisons between Belay et al 53,60 report about MIS-A and previous surveillance data about MIS-C. Compared to patients with MIS-C (n = 1733) from previously published surveillance data, a higher proportion of the patients with MIS-A (n = 20) had shock (75% vs. 37%), cardiac dysfunction (75% vs. 31%), myocarditis (70% vs. 17%), and pericardial effusion (45% vs. 23%).…”

“…Geographical distribution of reported cases of multisystem inflammatory syndrome in adults (as of October 26, 2022) 16,27–43 …”

“…[15][16][17]19,25,26 Many countries, including the United States, the UK, Italy, Israel, India, Canada, Japan, Pakistan, the United Arab Emirates, Croatia, Norway, France, Spain, Belgium, Portugal, South Africa, Lebanon, Switzerland, Thailand, Turkey, Austria, Cyprus, Sweden, Qatar, Oman, Brazil, South Korea, Germany, Uruguay, Mexico, Ireland, Malaysia, Australia, and Taiwan had reported the occurrence of MIS-A (Figure 1). 16,[27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43] To help understand this rare but fatal complication, this review was conducted to provide updated and comprehensive information on MIS-A.…”