Intracranial and Extracranial Neurovascular Manifestations of Takayasu Arteritis

Abstract

Background and purpose: Takayasu arteritis is a rare, large-vessel vasculitis that presents with symptoms related to end-organ ischemia. While the extracranial neurovascular manifestations of Takayasu arteritis are well-established, little is known regarding the intracranial manifestations. In this study, we characterize the intracranial and cervical neurovascular radiologic findings in patients with Takayasu arteritis.

Materials and methods: Patients with Takayasu arteritis who presented to our institution between 2001 and 2016 with intracranial and/or cervical vascular imaging were included in this study. Images were evaluated for the presence of vascular abnormalities, including intracranial or extracranial stenosis, vessel-wall thickening, dissection, subclavian steal, aneurysms, infarcts, and hemorrhages. Descriptive analyses are reported.

Results: Seventy-nine patients with Takayasu arteritis met the criteria for inclusion in this study. The most common presenting neurologic symptoms were headache (32.9%) and dizziness (15.2%). Intracranial and extracranial vascular imaging was performed in 84.8% and 89.9% of patients, respectively. Among patients with intracranial vascular imaging, 3 (3.9%) had intracranial aneurysms, 3 (3.9%) had acute large-vessel occlusion, 6 (7.6%) had intracranial vasculitis, and 1 (1.3%) had reversible cerebrovascular constriction syndrome. Among patients with cervical vascular imaging, 42 (53.1%) had some degree of narrowing of the common carotid artery and 18 (22.8%) had narrowing of the ICAs. Seventeen patients (23.6%) had subclavian steal.

Conclusions: Intracranial vascular abnormalities in patients with Takayasu arteritis presenting with neurologic symptoms are not rare, with cerebral vasculitis seen in 7.8% of patients, and stroke secondary to large-vessel occlusion, in 3.9% of patients. Cervical vascular manifestations of Takayasu arteritis were present in most patients in our study.

Fig 1.

A 36-year-old woman who presented with acute-onset left-sided hemiparesis. A, Noncontrast CT demonstrates a calcified embolus (white arrow) in the right ICA terminus along with loss of gray-white differentiation of the right basal ganglia. B, Diffusion-weighted MR imaging demonstrates a large right hemispheric infarct. C, CTA confirms a calcified embolus at the ICA termination. D, An arch aortogram demonstrates occlusion of the left common carotid artery origin (black arrow) along with multifocal narrowing of the right subclavian artery and right common carotid artery. There is occlusion of the left subclavian artery distal to its origin. E, MRA performed 12 months following the initial presentation shows chronic near-occlusion of the ICA terminus. F, Right common carotid artery cerebral angiogram shows a filling defect at the MCA origin, consistent with the now-chronic calcified embolus.

Fig 2.

A 49-year-old woman with sudden-onset left sensorineural hearing loss. A, CTA of the thoracic aorta and abdominal aorta demonstrates stenosis of the bilateral subclavian arteries, descending thoracic aorta, celiac and superior mesenteric artery origins, and the bilateral renal arteries. B, MRA at the time of presentation with sensorineural hearing loss shows an occluded basilar artery (white arrow) with no stenosis of the supraclinoid ICAs. MRA 1 year later shows high-grade stenosis of the bilateral ICA termini (C, white arrows) and persistent occlusion of the basilar artery (D), with new stenosis (black arrow) of the left posterior cerebral artery. She had no intervening stroke during this time.

Fig 3.

A 52-year-old woman who presented with subacute left-sided weakness. A, T2/FLAIR MR imaging demonstrates confluent areas of high T2 signal throughout the subcortical periventricular white matter of both hemispheres, consistent with chronic ischemic changes with encephalomalacia on the right. There were also chronic infarcts in the bilateral thalami and left pons (not shown). B, Left vertebral artery cerebral angiogram demonstrates diffuse mild irregularity of the basilar artery. Left ICA (C) and right ICA (D) cerebral angiograms in the lateral projection demonstrate multifocal areas of luminal narrowing in the distal territories of the anterior cerebral arteries, MCAs, and posterior cerebral arteries bilaterally, consistent with vasculitis.

Fig 4.

A 66-year-old woman with a nearly 20-year diagnosis of Takayasu arteritis with pan-aortic disease, bilateral common carotid artery involvement, and bilateral subclavian disease. A, The patient presented with acute headache and visual symptoms, and noncontrast CT demonstrates attenuated subarachnoid hemorrhage isolated to the right side of the prepontine and premedullary cisterns. B, Left vertebral artery cerebral angiogram demonstrates a focal outpouching of the distal intradural right vertebral artery (black arrow), with a focal stenosis just distal to the aneurysm. There was no PICA origin at this location because the PICA arose from a right AICA-PICA trunk. Also, note the prominent collaterals between the left vertebral artery and occipital artery secondary to long-term occlusion of the left external carotid artery in Takayasu arteritis (white arrows). C, The distal right vertebral artery was occluded with coils. D, Repeat left vertebral artery cerebral angiogram demonstrates occlusion of the intradural right vertebral artery.