Journal List > J Rheum Dis > v.20(3) > 1064043

Lee, Lee, Kang, Choi, Kim, Koo, So, Lee, and Yoo: A Case Report of Patient with Takayasu's Arteritis Complicated by Reversible Cerebral Vasoconstriction Syndrome

Abstract

Takayasu's arteritis (TA) is a chronic inflammatory vascular disease that mainly affects large vessels. Central nervous system involvement occurs in about 20% of cases with rare involvement of intracranial vessel, and its typical manifestation is cerebral ischemia or stroke. Reversible cerebral vasoconstriction syndrome (RCVS) is a group of disorders with prolonged, but reversible vasoconstriction of the cerebral arteries with acute-onset, severe, recurrent headaches with or without neurologic signs or symptoms. We report a case of TA in a 17-year old girl who presented with secondary RCVS. She complained of thunderclap headache, seizure and acute stroke. 3-dimensional computed tomography scan and magnetic resonance angiography of head revealed irregular thickening of aortic wall and its main branches with multifocal narrowing of intracranial basilar artery, which improved after oral nimodipine intake. This case highlights RCVS as an unusual manifestation of TA and demonstrates the diagnosis, treatment and response to therapy of RCVS in TA, which resolved after treatment with calcium channel blocker.

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Figure 1.
Diffusion weighted image of brain MRI shows high signal intensity at bilateral occipital lobes and right frontal lobe (arrows).
jrd-20-194f1.tif
Figure 2.
3D CT scan of aorta shows obstruction of bilateral subclavian arteries (arrows).
jrd-20-194f2.tif
Figure 3.
MR angiography (A). Multifocal segmental narrowing of basilar artery (B). Previously shown multifocal segmental narrowing of basilar artery (white arrows) was improved (black arrow) after 2 weeks of 3rd corticosteroid pulse therapy.
jrd-20-194f3.tif
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