Paediatric Moyamoya disease: acute presentation with fever and confusion in an 8-year-old: a case report

Abstract

Introduction and importance: Moyamoya is a chronic vaso-occlusive cerebrovascular disorder which involves internal carotid artery and its proximal branches, forming compensatory Moyamoya vessels. It may manifest with diverse symptoms, but early detection is crucial for a favourable prognosis. Case presentation: The authors present a case of an 8-year-old child who presented to the emergency with acute onset fever and confusion in speech. Although the symptoms were vague, she was advised for a thorough investigation. MRI of the brain revealed an infarct on the brain that raised suspicion of a vaso-occlussive disorder. Subsequent magnetic resonance angiography and digital subtraction angiography revealed underlying Moyamoya disease. Clinical discussion: Although the initial diagnosis of Moyamoya disease can be challenging, prompt diagnosis and simple medical measures like single antiplatelet regimens are useful for secondary ischaemic prevention. Conclusion: This case highlights the essence of considering Moyamoya disease as one of the differentials while dealing with children presenting with subtle cerebrovascular symptoms.