A Practical Approach to Headache in Moyamoya Disease

Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular disorder of the intracranial internal carotid arteries characterized by fragile collateral vessel formation. Although ischemic and hemorrhagic strokes are the most widely recognized manifestations of MMD, headaches are common, often disabling, and remain underacknowledged. Epidemiological studies report headache in 17%–85% of MMD patients, with particularly high rates among pediatric patients. Clinically, headache phenotypes are diverse and include migraine-like headaches with or without aura, tension-type, cluster, and hemiplegic variants. These presentations often overlap with primary headache disorders, complicating the diagnosis and sometimes delaying the recognition of underlying MMD. The pathophysiology of MMD-related headaches is multifactorial, involving vascular stenosis, abnormal collateral circulation, altered hemodynamics, and neurogenic inflammation. Chronic hypoperfusion may lower the threshold for cortical spreading depression, contributing to migraine-like or aura-associated symptoms. Surgical revascularization has been reported to alleviate headaches in both pediatric and adult patients, but persistent or new headaches may occur postoperatively, and long-term outcomes remain inconsistent. Management often involves general analgesics such as acetaminophen and non-steroidal anti-inflammatory drugs, but vasoconstrictive agents (e.g., triptans and ergotamines) should be avoided. Lasmiditan, a non-vasoconstrictive 5-HT1F agonist, may represent a safer option for acute treatment, while the efficacy of other pharmacological agents remains unclear due to limited evidence. In conclusion, headaches in MMD are not only a frequent source of disability but also a potential clinical marker of disease activity. Wider recognition of their epidemiology, phenotypes, and mechanisms may improve the diagnosis, guide individualized treatment, and ultimately enhance quality of life for patients.