[Three Cases of Moyamoya Disease with a History of Kawasaki Disease]

Area of Science:

• Neurology
• Pediatrics
• Vascular Surgery

Background:

• Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by stenosis or occlusion of the terminal internal carotid arteries (ICAs) and the development of collateral vessels.
• Kawasaki disease is an acute febrile illness that can affect blood vessels, including coronary arteries, but its association with moyamoya disease is less commonly reported.

Observation:

• Three pediatric and adult cases are presented, all with a documented history of Kawasaki disease treated with immunoglobulin or aspirin.
• Patients presented with varying neurological symptoms, including headache, lisping, and epilepsy, alongside diagnosed moyamoya disease.
• Diagnostic imaging revealed stenotic lesions in the ICAs, with some cases showing progression of stenosis and cerebral blood flow impairment over time.

Findings:

• The cases suggest a potential association between Kawasaki disease and the development of moyamoya disease, particularly in individuals with a history of this childhood illness.
• Despite initial mild or absent ischemic lesions on MRI, angiography confirmed significant ICA stenosis in all reported cases.
• Surgical interventions, including encephaloduroarteriosynangiosis and bilateral direct bypass, were performed in response to worsening symptoms and progressing disease.

Implications:

• This report underscores the importance of considering moyamoya disease in patients with a history of Kawasaki disease, especially those presenting with neurological deficits.
• Further research is warranted to elucidate the underlying mechanisms connecting Kawasaki disease and moyamoya disease.
• Enhanced surveillance and timely neurovascular assessment may be beneficial for individuals with a history of Kawasaki disease to detect and manage moyamoya disease effectively.