Subclavian steal syndrome secondary to takayasu arteritis in a caucasian stroke patient

Abstract

Introduction: Takayasu Arteritis (TA) is a chronic, large-artery vasculitis of unknown etiology, with a higher incidence in Asians that predominantly affects the aorta and its main branches. Severe stenosis or occlusion of the subclavian artery (SA, involved in up to 85% of TA cases) may cause subclavian steal syndrome (SSS) manifesting with symptoms of brainstem Ischemia. In Asia, as many as 36% of patients undergoing surgical management of SSS have TA as the underlying etiology. Case description: A 26-year-old, right-handed Caucasian woman, with a 4-year history of TA treated with oral corticosteroids on a daily basis, presented with recurrent, transient episodes of dizziness, gait imbalance and perioral numbness during the past three months. Her symptoms lasted for minutes and were provoked by physical activity (floor-sweeping, window-cleaning). Physical examination disclosed absent right brachial-artery pulses, a 32mmHg-difference in SBP (systolic blood pressure) between the right (92mmHg) and left (125mmHg) arm and the presence of bruit over the descending-aorta. Carotid Duplex and Transcranial Doppler studies disclosed the presence of a right subclavian steal phenomenon with the left vertebral artery (VA) providing blood to the posterior circulation as well as to the right arm via the right VA. Aortic-arch-MRA showed a right SA occlusion and stenosis of the descending-aorta. Delayed retrograde filling of right VA was documented following contrast injection. Conclusion: Nonatherosclerotic etiologies of SSS are rare in whites, in which atherosclerosis is the most common cause of SSS. The present case underscores that despite the very low incidence of TA in Caucasians (0.8/1.000.000), this large-vessel vasculitis of unknown etiology should always be considered in the differential diagnosis of subclavian steal syndrome in Caucasian women aged less than 40 years.