Cerebral vasculitis in severe Kawasaki disease: early detection by magnetic resonance imaging and good outcome after intensive treatment

Abstract

Kawasaki disease is an acute vasculitis, that has a classic complication of acquired coronary artery aneurysm. Severe forms with multi‐organ involvement or neurological dysfunction are rare. Cerebral vascular involvement has been related to large‐vessel injury or cardioembolism, leading to focal brain infarction. A 4‐year‐old female presented with unusual, rapidly catastrophic Kawasaki disease with refractory shock, acute renal failure, and coma, requiring intensive haemodynamic management. The observation of diffuse micro‐haemorrhages (T2*‐weighted sequence) associated with white matter injury on brain magnetic resonance imaging (MRI) pointed towards lesions of the medium/small blood vessels. Cerebral vasculitis was suspected and the immunosuppressive treatment was increased Subsequently, the patient’s recovery was rapid. On follow‐up severe, bilateral vitritis was evident and surgery improved visual outcome. Early recognition of severe or unusual forms of Kawasaki disease could lead to more favourable outcome using appropriate treatment strategies. Diffuse cerebral micro‐haemorrhages on T2* brain MRI sequences might be a key sign for the diagnosis of medium or small cerebral vessel involvement.

Cognitive Development After Kawasaki Disease ― Clinical Study and Validation Using a Nationwide Population-Based Cohort ―

“…Nevertheless, some studies have suggested that multisystemic vasculitis in children with KD may result in abnormalities in blood perfusion and inflammatory changes in the brain. 12 Other studies have reported that KD patients have behavioral sequelae following KD, 32 as well as neurological abnormalities in both generalized and localized CNS symptoms. 14 However, a study conducted in the US suggested that the incidence of attentional, behavioral, or learning problems in KD patients did not differ to that in the general population.…”

“…17 Furthermore, coma and white matter injuries on brain magnetic resonance imaging (MRI) have been observed in KD patients. 12 Nevertheless, few studies have focused on whether KD is related to cognitive impairment sequelae. 18, 19 One study conducted in Ottawa, Canada, recruited 32 KD patients aged 4-18 years, with their siblings enrolled as control subjects.…”

Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease

“…47 One important characteristic of KDSS is the multiorgan nature of the disease: carotid artery dilatation, mesenteric and splenic vasculitis and cerebral infarcts were found in this group of patients. 18,27,28,30 It is interesting to note that the majority of patients with KDSS presented with abdominal symptoms: gastrointestinal symptoms included gastrointestinal bleeding and intestinal perforation. 19 On two autopsies, along with the coronary artery involvement, vasculitis in the mesenteric arteries was also found.…”

Sorting nexin 24 genetic variation associates with coronary artery aneurysm severity in Kawasaki disease patients

“…It is believed to be caused by infectious agents, host immune dysregulation, and genetic susceptibility. During the acute stage of KD, activation of vascular endothelial cells and increased serum levels of proinflammatory cytokines lead to inflammation and injury of blood vessels [6-8]. The vascular inflammation may induce the development of aneurysms and cardiac complications.…”