Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain . Clinical presentation of moyamoya disease includes transient ischemic attack (TIA), cerebral infarction, intracerebral hemorrhage, and seizure . TIA is one of the most common clinical presentations of moyamoya disease both in pediatric and adult cases , while intracerebral hemorrhage is mostly seen among adult cases . Recently, headache is also considered to be one of the common clinical presentations of moyamoya disease. Patients with moyamoya disease may complain of headache before and after revascularization surgery. In this chapter, the authors focus on headache as the clinical presentation of moyamoya disease, and discuss the incidence and mechanism of this symptom. We further discuss the efficacy of revascularization surgery for the improvement of headache.
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