INCREASE ARTERIAL STIFFNESS IN MOYAMOYA DISEASE PRESENTING WITH HYPERTENSION AND STROKE: A NOVEL FINDING IN A VERY RARE CASE IN SINGAPORE

Abstract

Objective: Arterial stiffness in Moyamoya disease (MMD) has not been described before in literature. We describe a young 42 year-old lady who initially presented as hypertensive urgency and recurrent stroke and was later diagnosed with MMD. We would like to highlight increased arterial stiffness as a cause of hypertension in patients with MMD. Design and method: This is a case report of a 42 year old lady 42 year Singaporean lady presented in 2015 with headache and transient ischemic attack(TIA) and systolic blood pressure was 200mmHg. She was diagnosed with hypertension at the age 28 years and started on telmisartan 40 mg OD. There was no end organ damage and secondary hypertension work up was negative. She has recurrent TIAs and was started on T. atorvastatin 40 mg ON and T clopidogrel 75 mg OD in Feb 2017. She presented with stroke in March 2019, and MRI revealed left posterior temporal-parietal infarct, MRA revealed severe stenosis of left terminal internal carotid artery(ICA). She presented with recurrent stroke in April 2019 and MRI/MRA revealed scattered acute infarcts in left MCA and ACA territories and severe steno-occlusive disease involving left ICA. She underwent left external carotid –internal carotid bypass in June 2019. In light of these findings, MMD was diagnosed. Results: She was started on T spironolactone and her blood pressure was kept in stable range of 130–150/80–95mmHg. An arterial stiffness study by SphygmoCor in June 2020 showed markedly increased arterial stiffness with markedly increased augmentation index, aortic augmentation pressure, aortic systolic pressure and aortic pulse pressure for the patient’s age. Conclusions: We present an interesting and rare case of hypertension in a young patient with MMD associated with increased arterial stiffness, with no renovascular disease. This is one of the first cases reported in MMD with increased arterial stiffness. We also highlighted genetic studies and case reports that have suggested that the histopathological process underlying Moyamoya vessels may also affect systemic vessels. We hope that this article will inspire more research on the relationship between MMD and hypertension, particularly in terms of how systemic vasculature is affected.