Thyrotoxic Graves’ Disease and Moyamoya-like Vasculopathy in Latino Patients: A Case Series (P3.113)

INTRODUCTION Graves’ disease, an autoimmune hyperthyroid state, has been reported with occlusive intracranial arteriopathy of the terminal ICA. Moyamoya syndrome is a cerebral vasculopathy characterized by stenosis of the terminal portion of the ICA primarily and development of a fine collateral network of vessels. It has been reported to be more common in people of Eastern Asian descent and to date has not been reported in Latino patients. Recently, there has been more evidence supporting the association of Moyamoya angiographic states with Graves’ disease. However, it is unclear whether Grave’s with thyrotoxicosis causes arteriopathy due to a hyperadrenergic state or due to inflammation. METHODS AND RESULTS: We describe five cases of patients with Graves’ disease in the setting of thyrotoxicosis presenting with ischemic strokes. Workup revealed radiological and clinical findings consistent with a Moyamoya-like pathogenic process. All patients survived their acute cerebral ischemia and treatment with immunotherapy yielded variable results possibly due to lack of inflammation. CONCLUSION: Much remains undiscovered regarding the association between Graves' disease and Moyamoya-like vasculopathy especially with regard to the mechanism of ICA stenosis. Prevention and screening with ultrasound guided modalities to date has not been performed for which we will be initiating a prospective trial. Treatment with immunotherapy yield modest benefits, perhaps due to the main etiology of fibrosis being from a hyperadrenergic state.