PS-R06-5: CASE REPORT OF INCREASED ARTERIAL STIFFNESS IN MOYAMOYA DISEASE IN A YOUNG CHINESE PATIENT PRESENTING AS RECURRENT STROKE IN SINGAPORE

Abstract

Introduction: Moyamoya disease (MMD) is characterised by progressive stenosis of distal ICA and the resulting hazy network of basal collateral vessels. Increase arterial stiffness in MMD is not well described. Case report: We describe a young patient ML, a 42-year-old Chinese female, presented to hospital in June 2015 with headaches, on a background of known hypertension diagnosed at the age of 28 years. Her systemic examination was normal. Investigations were unremarkable. She was followed in General Medicine (GM) clinic from 2015, which ruled out secondary causes of hypertension, including renovascular disease, renal artery stenosis, phaeochromocytoma and hyperthyroidism. ML was treated for presumptive essential hypertension. Over time, her BP remained stable on candesartan 16 mg and bisoprolol 2.5 mg once daily. She continued to have intermittent headaches. Recurrent ischaemic strokes and diagnosis of Moyamoya disease ML presented with intermittent right-sided facial numbness in February 2017. A magnetic resonance imaging (MRI) brain done showed no acute infarct or hemorrhage, but moderately severe narrowing of the left terminal internal carotid artery (ICA), associated with poor flow signals in both the A1 segment of the left anterior cerebral artery (ACA) and the M1 segment of the left middle cerebral artery (MCA). She was started on clopidogrel 75 mg and atorvastatin 40 mg daily. The second stroke occurred in March 2019, when ML presented with acute right upper and lower limb weakness, and right facial, upper and lower limb numbness. Magnetic resonance angiography (MRA) demonstrated severe stenosis at the left terminal ICA, also involving the left M1 and A1 segments; collateral flow was observed. The third stroke happened in April 2019, presenting with acute right-sided facial and upper limb weakness, with BP of 201/86mmHg. MRI and MRA of the brain revealed new acute infarcts scattered in the territories of the left MCA and ACA. A CT angiogram re-demonstrated severe steno-occlusive disease involving the left ICA T-junction and left M1 and A1 segments, with extensive basal collaterals. In light of these findings, Moyamoya disease (MMD) was diagnosed. ML underwent a left external carotid-internal carotid bypass in June 2019. An arterial stiffness study by syphgmoCor in June 2020 using markedly increased arterial stiffness. Conclusion: We present first and very rare case of hypertension in a young patient with MMD associated with increased arterial stiffness. Systemic vascular affection in MMD may be the cause of increased arterial stiffness.