Abstract

Background and aim: Significant headache is often reported by patients with moyamoya arteriopathy (MMA) but has a poorly understood pathophysiology. To decide on management, physicians often focus on stroke-like symptoms while the impact on quality of life and economic burden of chronic headache is often overlooked. We aimed to investigate the effect of revascularization surgery on headache in MMA and potential association with cerebrovascular reactivity (CVR). Methods: This was a single-center retrospective study in adult patients with MMA, who underwent extracranial-intracranial (EC-IC) bypass surgery between Jan 2010 - Sept 2022 and had CVR-MRI performed. Clinical and imaging data were collected through systematic chart review complemented by structured headache questionnaires. We assessed the association between headache and CVR before and after revascularization surgery along with potential predictors using logistic regression analysis. Results: Fifty-nine patients were included (mean age 44±13 years, 42 women (71%)). Thirty-seven patients (63%) had bilateral MMA of whom 19 (51%) had bilateral EC-IC bypass surgery. Thirty-eight patients (64%) reported headache pre-surgery with mostly migraine-like features. More than a third of patients (37%) lost workdays or quit their job because of headache. Headache improved in 26/38 (68%) patients post-surgery with a reduction in pain severity (median VAS 6 to 3; p = 0.005) and sick leave (37% to 8%; p<0.001). CVR improved in 33/43 (77%) patients who had pre- and post-surgery CVR-MRI, which was associated with headache improvement (OR:14; 95% CI:1.4-131). Pre-operative headache severity (VAS-score) was an independent predictor for headache improvement post-surgery (OR:1.7; 95% CI:1.2-2.5). Conclusions: Results of this study showed improvement of headache in most patients with MMA after revascularization surgery and association with improvement in CVR, supporting the hypothesis of a vascular origin. Future larger cohort studies should confirm this hypothesis, improve understanding of underlying pathophysiology and identify potential future treatment targets to guide management for this underappreciated yet disabling aspect of MMA.

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