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

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.

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.

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.

This case highlights the essence of considering Moyamoya disease as one of the differentials while dealing with children presenting with subtle cerebrovascular symptoms.