Evaluation and treatment of headache associated with moyamoya disease – a narrative review

Abstract

Background Headache in patients with moyamoya disease is an under-addressed topic in the medical literature. Delay in the diagnosis of moyamoya disease or inappropriate treatment of headache could lead to devastating cerebrovascular outcome. With the evolving understanding of moyamoya disease, migraine pathophysiology, and various migraine-specific medications that have become available, it is crucial to provide an updated overview on this topic. Methods We searched PubMed for keywords including moyamoya disease, moyamoya syndrome, headache in moyamoya, surgical revascularization, surgical bypass, migraine and moyamoya, and calcitonin gene-related peptide (CGRP). We summarized the literature and provide a comprehensive review of the headache presentation, possible mechanisms, the impact of various surgical revascularizations on headache in patients with moyamoya disease, and the medical management of headache incorporating novel migraine-specific treatments. Results and conclusion: The most common headache phenotype is migraine; tension-type headache, hemiplegic migraine, and cluster headache have also been reported. Most patients experience improvement of headache after surgical revascularization, though some patients report worsening, or new-onset headache after surgery. Given the complexity of moyamoya disease, careful consideration of different types of medical therapy for headache is necessary to improve the quality of life while not increasing the risk of adverse cerebrovascular events. More prospective studies are warranted to better understand and manage headache in patients with moyamoya disease.

Adult Moyamoya Disease and Syndrome: Current Perspectives and Future Directions: A Scientific Statement From the American Heart Association/American Stroke Association

“…37 In addition, headaches are highly pervasive, most often manifesting with migraine-like features, but symptoms may resemble other headache subtypes. 38 Last, moyamoya vasculopathy can be associated with other diseases such as congenital and autoimmune syndromes, and patients may present with complications from those underlying diseases. 39 They should be assessed accordingly, because their management may complicate the treatment of the cerebrovascular components of the disease.…”

“…should be avoided. 33,38 In patients undergoing surgical revascularization, postoperative improvement of headache is common, but postsurgical worsening or new onset of headache has also been reported. 33,38 Considerations for Clinical Practice…”

“…84,85 The pathophysiological mechanisms and optimal treatment of headache in moyamoya vasculopathy are uncertain, but medications that limit vasodilation (calcitonin gene-related peptide–targeted therapies), enhance vasoconstriction (triptans, ergots), or lower blood pressure (β-blockers, calcium channel blockers) should be avoided. 33,38 In patients undergoing surgical revascularization, postoperative improvement of headache is common, but postsurgical worsening or new onset of headache has also been reported. 33,38…”

Acute Treatment of Headache (Focus on Migraine)

“…However, more studies are needed to establish the safety of patients with high risk of cerebrovascular events, due to the concern that CGRPtargeting therapies could potentially affect the compensatory vasodilation in the setting of acute ischemia. [39][40][41]…”

“…45 Therefore, it can be considered for patients with contraindications to triptans or ergots, including history of ischemic stroke or coronary artery disease. 41 Of note, lasmiditan is currently listed a Category-V controlled substance. In a human abuse potential study conducted on 58 recreational drug abusers, lasmiditan had a higher “drug-liking” score compared with placebo, indicating a potential for abuse, though the score was significantly lower when compared with alprazolam.…”