Progressive Vertebrobasilar Vasculopathy and Stroke Secondary to Giant Cell Arteritis

HomeStrokeVol. 53, No. 9Progressive Vertebrobasilar Vasculopathy and Stroke Secondary to Giant Cell Arteritis Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessCase ReportPDF/EPUBProgressive Vertebrobasilar Vasculopathy and Stroke Secondary to Giant Cell Arteritis Yasamin Mahjoub, Erica D. McKenzie, Saher Raouf and Mohammed Almekhlafi Yasamin MahjoubYasamin Mahjoub Correspondence to: Yasamin Mahjoub, MD, Foothills Medical Centre, 1403 29 St NW, Calgary, AB T2N 2T9. Email E-mail Address: [email protected] https://orcid.org/0000-0003-0898-3307 Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Alberta, Canada. , Erica D. McKenzieErica D. McKenzie https://orcid.org/0000-0003-4369-413X Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Alberta, Canada. , Saher RaoufSaher Raouf https://orcid.org/0000-0003-3338-7937 Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Alberta, Canada. and Mohammed AlmekhlafiMohammed Almekhlafi https://orcid.org/0000-0001-9550-8197 Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Alberta, Canada. Originally published15 Aug 2022https://doi.org/10.1161/STROKEAHA.122.039503Stroke. 2022;53:e435–e438Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: August 15, 2022: Ahead of Print Take-Home PointsGiant cell arteritis is a rare cause of large artery stroke that is important to recognize given treatment implications and significant morbidity and mortality.Strokes associated with giant cell arteritis tend to preferentially affect the vertebrobasilar artery territories.Temporal artery biopsy is the gold standard for diagnosis of giant cell arteritis.Imaging can aid in the diagnosis of giant cell arteritis, including the "halo sign" on duplex ultrasonography, vessel wall enhancement on magnetic resonance imaging, and vessel thickening or blurring on CT/magnetic resonance angiography.Treatment of giant cell arteritis consists of immediate high-dose glucocorticoids, followed by tocilizumab or methotrexate for refractory or relapsing disease.An 80-year-old man presented to the emergency department with recurrent episodes of imbalance. His history was significant for diabetes, prior smoking, and a remote nephrectomy for renal cell carcinoma.Six months before presentation to our hospital, he had developed gradual-onset imbalance. Six weeks before his presentation, outpatient computed tomography angiogram neck revealed multiple foci of stenosis in both vertebral arteries and the basilar artery (Figure 1). Two weeks before his presentation, he was admitted to a peripheral hospital with transient, sudden-onset imbalance, blurred vision, and slurred speech, lasting for several minutes. Magnetic resonance imaging brain revealed multifocal cerebellar infarcts and occlusion of the V3/V4 segment of the left vertebral artery. Bradycardia and atrial flutter were identified and apixaban was started.Download figureDownload PowerPointFigure 1. Computed tomography (CT) angiography of the vertebrobasilar system 6 wk apart. CT angiography of the vertebrobasilar system 6 wk before presentation (A–C) and at presentation (D–F). Coronal thin maximal intensity projection (MIP) images show focal narrowing at the basilar tip and origin of the posterior cerebral arteries (A), which evolved to near-occlusion at presentation (D). The caliber of the left vertebral artery (B) at the level of C3 shows marked narrowing at presentation (E). The left vertebral artery (C) shows diminutive caliber throughout its extracranial course at presentation (F) and is occluded distally (not shown).He presented to our hospital with a recurrent episode of sudden-onset imbalance leading to collapse without loss of consciousness. His symptoms resolved before stroke team assessment, when he had an essentially normal neurological examination. Head and neck computed tomography angiogram revealed progressive stenosis involving the left vertebral artery, basilar tip, and posterior cerebral artery origins (Figure 1).On further history, he described occasional blurred vision, bilateral temporal headaches, hardening of the temporal arteries, jaw claudication, and new-onset Raynaud's phenomenon in the left fingers over the past year. He had no myalgias/arthralgias, muscle weakness, weight loss, fevers, or skin changes. On palpation, the temporal arteries were prominent and nodular but nontender.CRP (C-reactive protein) was modestly elevated at 19.2 mg/L (reference range 0–8.9 mg/L) while erythrocyte sedimentation rate (ESR) was normal for age: 26 mm/h (reference range 0–10 mm/h). 3T magnetic resonance imaging showed multifocal subacute infarcts of both cerebellar hemispheres and intrinsic T1 hyperintensity of a heterogeneously thickened distal basilar wall (Figure 2). Enhanced MR angiography demonstrated enhancement of the vertebral, basilar, and superficial temporal arteries. The anterior circulation was normal.Download figureDownload PowerPointFigure 2. Magnetic resonance imaging (MRI) and MR angiography at presentation. Axial T2 FLAIR (A) shows multifocal subacute infarcts of both cerebellar hemispheres. Angiography (B) shows occlusion of the left vertebral artery and narrowing of the distal basilar artery. Post-gadolinium coronal views show vertebral (C), basilar (D), and superficial temporal (E) artery wall enhancement (arrows).Temporal artery biopsy showed focal vessel occlusion, multiple segments of high-grade stenosis, and extensive mural thickening with mixed inflammation, fibrosis, and focal necrosis. There were giant cells scattered in all vessel layers. Fibrinoid necrosis was present and the internal elastic layer was circumferentially disrupted. These findings confirmed active vasculitis consistent with giant cell arteritis (GCA). MR aortogram revealed mild irregularity of the infrarenal abdominal aorta and right common iliac artery, favored secondary to atherosclerosis rather than vasculitis.He was diagnosed with GCA and started on steroids. Tocilizumab was later initiated by rheumatology.Giant cell arteritis is a medium- and large-vessel vasculitis mainly affecting the aorta and its branches, including the carotid and vertebral arteries. Inflammation in GCA can cause vessel scarring, stenosis, and occlusion with resultant ischemic complications.1 Classic symptoms are headache, jaw claudication, scalp tenderness, and vision changes (notably, monocular vision loss).1 However, patients may present with polymyalgia symptoms or nonspecific constitutional symptoms including fatigue, fever, and weight loss.1 GCA occurs almost exclusively after 50 years of age, with peak incidence between 70 and 79 years.1 Pathology shows granulomatous inflammation of the internal elastic lamina2 resulting in necrotizing arteritis.Stroke is a rare complication of GCA, with a reported prevalence of 2% to 7% in most series.1 Distinguishing GCA from traditional vascular etiologies remains a diagnostic challenge due to overlapping demographics and clinical-radiological presentations. Infarcts from GCA preferentially affect the vertebrobasilar arteries, based on several case series.1 Which vertebral artery segments demonstrate stenosis or occlusion may help differentiate GCA from atherosclerosis: GCA most often involves V3 and proximal V4 segments (≤5 mm distal to the dural perforation), while atherosclerosis most commonly affects the vertebral artery origin (V1) and distal V4 segments.2 The intradural vertebral artery is thought to be spared by GCA due to the lack of arterial wall elastic tissue distal to the dural perforation.2The American College of Rheumatology outline five classification criteria for GCA: localized headaches, age ≥50 years, ESR ≥50 mm/hr, decreased temporal artery pulse, and temporal artery biopsy indicating inflammation with or without multinucleated giant cells.1 Presence of 3/5 criteria yielded a sensitivity of 93.5% and specificity of 91.2% in the original publication.1 CRP is more sensitive than ESR among those referred for temporal artery biopsy.3 The Table summarizes the diagnostic performance of GCA biomarkers.Table. Performance of Selected Biomarkers Compared With Clinical Diagnosis of GCADiagnostic testSensitivitySpecificityESR66%357%3CRP67%368%3High-resolution MR of cranial arteries73%488%4Halo sign on ultrasound67%595%5Values for ESR and CRP are derived from a study of a population referred for temporal artery biopsy. CRP indicates C-reactive protein; ESR, erythrocyte sedimentation rate; and GCA, giant cell arteritis.Importantly, normal inflammatory markers do not rule out GCA; normal ESR and CRP has been reported in 4% of biopsy-proven cases.6 Lower ESR and CRP levels have been reported in patients with GCA with stroke compared with GCA alone, potentially obscuring the diagnosis.7Neuroimaging is a valuable adjuvant in GCA diagnosis. Duplex ultrasound is relatively low-cost and noninvasive. The presence of a "halo sign" (noncompressible, hypoechoic, mostly concentric arterial wall thickening) in the temporal arteries can suggest GCA.1 While vertebral arteries are not routinely assessed with duplex ultrasound, the presence of a vertebral halo sign may suggest ischemic stroke.1 Blurred superficial temporal artery wall margins and perivascular enhancement on computed tomography angiogram can aid in GCA diagnosis.8 High-resolution contrast-enhanced magnetic resonance imaging may show arterial wall thickening and enhancement.4 These specialized techniques require expert interpretation for GCA diagnosis and findings may not be reported routinely in studies ordered for stroke workup. Aortic damage has been reported in 15% to 23% of patients with GCA in computed tomography angiogram studies, and additional body imaging is typically performed to evaluate for aortic aneurysm or occlusive disease.9The 2018 European League Against Rheumatism recommendations for treatment of large-vessel vasculitis suggest immediate high-dose glucocorticoids (ie, 40–60 mg/day prednisone equivalent) for GCA. Adjunctive tocilizumab or methotrexate is recommended in refractory or relapsing disease and those at elevated risk of adverse effects from long-term glucocorticoids.10 While retrospective studies suggested antiplatelet and anticoagulant agents may protect against ischemic events in GCA, a subsequent meta-analysis did not show a significant effect.1 These agents are not included in the 2018 European League Against Rheumatism recommendations unless used for alternative indications.10Vertebrobasilar vasculopathy is likely to be encountered at stroke referral centres and GCA should be considered as a potential etiology. This case highlights a scenario with classic vascular risk factors (diabetes, atrial fibrillation, smoking history, and age), where clinical and imaging features led to the correct diagnosis in the setting of posterior circulation strokes.Article InformationSources of FundingNone.Disclosures None.FootnotesCorrespondence to: Yasamin Mahjoub, MD, Foothills Medical Centre, 1403 29 St NW, Calgary, AB T2N 2T9. Email ymahjoub@ucalgary.caReferences1. Bajko Z, Balasa R, Maier S, Motataianu A, Barcutean L, Andone S, Stoian A, Filep RC. Stroke secondary to giant-cell arteritis: a literature review.Exp Ther Med. 2021; 22:876. doi: 10.3892/etm.2021.10308Google Scholar2. Rüegg S, Engelter S, Jeanneret C, Hetzel A, Probst A, Steck AJ, Lyrer P. Bilateral vertebral artery occlusion resulting from giant cell arteritis: report of 3 cases and review of the literature.Medicine (Baltimore). 2003; 82:1–12. doi: 10.1097/00005792-200301000-00001CrossrefMedlineGoogle Scholar3. Chan FLY, Lester S, Whittle SL, Hill CL. The utility of ESR, CRP and platelets in the diagnosis of GCA.BMC Rheumatol. 2019; 3:14. doi: 10.1186/s41927-019-0061-zGoogle Scholar4. Duftner C, Dejaco C, Sepriano A, Falzon L, Schmidt WA, Ramiro S. Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: a systematic literature review and meta-analysis informing the EULAR recommendations.RMD Open. 2018; 4:e000612. doi: 10.1136/rmdopen-2017-000612CrossrefMedlineGoogle Scholar5. Sebastian A, Coath F, Innes S, Jackson J, van der Geest KSM, Dasgupta B. Role of the halo sign in the assessment of giant cell arteritis: a systematic review and meta-analysis.Rheumatol Adv Pract. 2021; 5:rkab059. doi: 10.1093/rap/rkab059Google Scholar6. Kermani TA, Schmidt J, Crowson CS, Ytterberg SR, Hunder GG, Matteson EL, Warrington KJ. Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of giant cell arteritis.Semin Arthritis Rheum. 2012; 41:866–871. doi: 10.1016/j.semarthrit.2011.10.005Google Scholar7. De Boysson H, Liozon E, Larivière D, Samson M, Parienti JJ, Boutemy J, Maigné G, Silva NM, Ly K, Touzé E, et al. Giant cell arteritis–related stroke: a retrospective multicenter case-control study.J Rheumatol. 2017; 44:297–303. doi: 10.3899/jrheum.161033.CrossrefGoogle Scholar8. Conway R, Smyth AE, Kavanagh RG, O'Donohoe RL, Purcell Y, Heffernan EJ, Molloy ES, McNeill G, Killeen RP. Diagnostic utility of computed tomographic angiography in giant-cell arteritis.Stroke. 2018; 49:2233–2236. doi: 10.1161/STROKEAHA.118.021995LinkGoogle Scholar9. Koster MJ, Matteson EL, Warrington KJ. Large-vessel giant cell arteritis: diagnosis, monitoring and management.Rheumatology (Oxford). 2018; 57(suppl_2):ii32–ii42. doi: 10.1093/rheumatology/kex424CrossrefMedlineGoogle Scholar10. Hellmich B, Agueda A, Monti S, Buttgereit F, de Boysson H, Brouwer E, Cassie R, Cid MC, Dasgupta B, Dejaco C, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis.Ann Rheum Dis. 2020; 79:19–30. doi: 10.1136/annrheumdis-2019-215672CrossrefMedlineGoogle Scholar eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. 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