Efficacy of Revascularization Surgery for Moyamoya Syndrome Associated With Graves' Disease

Abstract

Appropriate management of moyamoya syndrome associated with Graves' disease is undetermined because of the rarity of this combination. Patients tend to present with cerebrovascular events such as transient ischemic attack (TIA) in a thyrotoxic state, which is relieved by proper antithyroid therapy. Four patients with moyamoya syndrome associated with Graves' disease were successfully treated with revascularization surgery on 5 hemispheres among 58 consecutive patients (2-62 years old, mean 34.4 years) with moyamoya disease in 80 hemispheres treated from March 2004 to May 2007. Three patients presented with TIA, and one patient presented with intracerebral hemorrhage. Three patients were thyrotoxic at the onset of the cerebrovascular events. All patients underwent revascularization surgery after normalization of thyroid function. Euthyroid state was strictly maintained perioperatively. One patient developed symptomatic cerebral hyperperfusion, which was resolved by blood pressure control. Postoperative courses of the other patients were uneventful, and all 4 patients have remained neurologically stable after discharge. Cerebrovascular reconstruction surgery is a successful treatment option for moyamoya syndrome associated with Graves' disease. Timing of surgery during the euthyroid state and perioperative management considering the thyroid function and the cerebral hemodynamic change are the keys to successful surgical treatment.

Identification of HLA-DRB1*04:10 allele as risk allele for Japanese moyamoya disease and its association with autoimmune thyroid disease: A case-control study

“…In this study, we demonstrated that the frequency of thyroid diseases, including GD and HT, is high in HLA-DRB1*04 : 10 -positive MMD patients compared with that in HLA-DRB1*04 : 10 -negative MMD patients, suggesting the relationship between the HLA-DRB1*04 : 10 haplotype and MMD and autoimmune thyroid diseases. Many studies have reported the association between thyroid dysfunction and MMD [7–12], suggesting that elevated autoantibody concentrations and hyperthyroidism are associated with stenotic lesions in the terminal portion of the internal carotid artery and aggressive MMD presentations [7–12]. However, the underlying mechanism of this association remains undetermined.…”

Acute Thrombectomy for Contralateral Internal Carotid Artery Occlusion after Revascularization Surgery for Quasi-moyamoya Disease: A Case Report

“…Endo et al reported the return of neurological symptoms associated with recurrent hyperthyroidism and discussed the efficacy of revascularization surgery. 16) Revascularization surgery appears acceptable for use when areas of vascular stenosis remain, despite adequate medication.…”

Fatal outcome in a Hispanic woman with moyamoya syndrome and Graves’ disease

“…There are reports of elevated levels of thyroid antibodies detected in some patients presenting with signs of ischemia secondary to moyamoya disease, even without impaired thyroid functionality (3). Given the increased presence of T-cells within affected vessels, the possibility for T-cell-mediated cross-reactivity between TSH receptor antibodies, and antigens within the cerebral arteries, as well as impaired T-cell function may be a common pathogenetic factor in both diseases (4, 5, 6, 7). Colleran et al (8) has elicited a positive correlation between elevated thyroxine levels with increased homocysteine and methylmalonic acid levels.…”

“…Cerebral oxygen consumption also increases, whereas cerebral perfusion decreases due to elevated sympathetic tone and subsequent vasoconstriction (5, 6, 1, 7). Smooth muscle and endothelium of vasculature are influenced by excess circulating thyroid hormones such that the combination of stiffened arteries seen in GD and the significant stenosis observed in MMD predisposes affected individuals to ischemic events and may even pose greater risk than either disease alone (5, 1, 9, 10). In other words, during marked hyperthyroid states, cerebral oxygen demand increases, whereas perfusion decreases and exceeds the compensatory mechanism offered by moyamoya collateral vessels.…”

“…(10) also describe a patient who had perioperative stroke-like syndrome during hyperthyroid state after revascularization surgery. Nonetheless, once euthyroid, surgical intervention currently remains the gold standard as 50% of those treated with antithyroid therapy relapse into hyperthyroid state (5). The only other reported fatality comes from Hsu et al .…”