Headache and focal neurologic signs following exposure to spicy aroma as an initial presentation of moyamoya disease

Moyamoya disease is a condition of the cerebrovascular system that involves stenosis of the intracranial internal carotid arteries as well as their proximal branches, often leading to stroke in affected patients. Here we describe the case of a patient with headache and focal neurologic signs following exposure to a spicy aroma, who initially had a negative vascular work-up and a preliminary diagnosis of a complex migraine syndrome. She subsequently developed infarction of the left frontal lobe, and imaging studies revealed the diagnosis of moyamoya disease. She was treated with an encephalodurosynangiosis procedure, with notable improvement. This case highlights the importance of considering moyamoya disease in the differential diagnosis of patients presenting with headaches with aura.