Endovascular Therapy for Intracranial Giant Cell Arteritis : Systematic Review, Technical Considerations and the Effect of Intra-arterial Calcium Channel Blockers

Abstract

Background: Giant cell arteritis (GCA) is a systemic vasculitis that may cause ischemic stroke. Rarely, GCA can present with aggressive intracranial stenoses, which are refractory to medical therapy. Endovascular treatment (EVT) is a possible rescue strategy to prevent ischemic complications in intracranial GCA but the safety and efficacy of EVT in this setting are not well-described.

Methods: A systematic literature review was performed to identify case reports and series with individual patient-level data describing EVT for intracranial GCA. The clinical course, therapeutic considerations, and technique of seven endovascular treatments in a single patient from the authors' experience are presented.

Results: The literature review identified 9 reports of 19 treatments, including percutaneous transluminal angioplasty (PTA) with or without stenting, in 14 patients (mean age 69.6 ± 6.3 years). Out of 12 patients 8 (66.7%) with sufficient data had > 1 pre-existing cardiovascular risk factor. All patients had infarction on MRI while on glucocorticoids and 7/14 (50%) progressed despite adjuvant immunosuppressive agents. Treatment was PTA alone in 15/19 (78.9%) cases and PTA + stent in 4/19 (21.1%). Repeat treatments were performed in 4/14 (28.6%) of patients (PTA-only). Non-flow limiting dissection was reported in 2/19 (10.5%) of treatments. The indications, technical details, and results of PTA are discussed in a single illustrative case. We report the novel use of intra-arterial calcium channel blocker infusion (verapamil) as adjuvant to PTA and as monotherapy, resulting in immediate improvement in cerebral blood flow.

Conclusion: Endovascular treatment, including PTA with or without stenting or calcium channel blocker infusion, may be effective therapies in medically refractory GCA with intracranial stenosis.

Keywords: Angioplasty; Cerebral ischemia; Intracranial stenosis; Vasculitis; Verapamil.

Fig. 1

Manifestations of giant cell arteritis (GCA) with large-vessel involvement. FDG-PET maximum intensity projection reconstruction (a) shows florid hypermetabolism throughout the cervical and subclavian axillary arteries (red arrows) as well as the aorta, iliac, and femoral vessels. Axial fusion PET-CT image of the same patient (b) shows disease activity in both carotid and vertebral arteries (white arrows). Anteroposterior angiography of the left subclavian artery (c) shows classical appearance of extracranial circumferential large artery stenosis (white arrow)

Fig. 2

Intracranial manifestations of giant cell arteritis with left ACA-MCA watershed ischemia on DWI MRI (a). Time-of-flight MR angiography showed high-grade stenosis of supraclinoid LICA (white curved arrow, b). CT perfusion showed at-risk parenchyma throughout the left hemisphere, shown as T_max map (c) which prompted urgent endovascular treatment (treatment #1). Pretreatment lateral projection LICA injection shows 2 sites of critical stenosis (d, white arrows) which were treated with submaximal balloon angioplasty (e). Posttreatment result f showed substantial improvement in angiographic transit time and return of antegrade flow to the ophthalmic and posterior communicating arteries. Careful review of images showed a tiny, non-flow limiting dissection measuring < 1 cm (red arrow)

Fig. 3

Calcium channel blocker infusion as monotherapy for intracranial giant cell arteritis. Pre-treatment angiography (lateral RICA projection) shows severe focal supraclinoid segment stenosis (a). Color-coded four-dimensional DSA (4D-DSA, b) shows prolonged transit time throughout the RICA circulation; sample velocity at the petrous segment time-to-peak (TTP) velocity of 4.53s. CTP performed the day prior to intervention showed at-risk tissue (prolonged T_max) throughout the right ICA territory (c, arrows). Post-verapamil infusion (20 mg, 15 min delay) angiogram is shown in d, with significant improvement in lumen diameter. Post-verapamil 4D-DSA (e) shows improved flow throughout the ICA distribution and normalization of TTP in the petrous segment (1.0s) (circle, labeled Ref). A CTP performed 10 h after verapamil infusion shows durable improvement in T_max in RICA distribution (f)