Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-22T13:01:03.926Z Has data issue: false hasContentIssue false

Chapter 21 - Cervical Artery Dissection and Cerebral Vasculitis

from Part V - Prevention

Published online by Cambridge University Press:  15 December 2020

By
Philippe A. Lyrer ,
Christopher Traenka  and
Stefan T. Engelter
Edited by
Jeffrey L. Saver  and
Graeme J. Hankey
Jeffrey L. Saver
Affiliation:
David Geffen School of Medicine, University of Ca
Graeme J. Hankey
Affiliation:
University of Western Australia, Perth
Get access

Summary

Cervical artery dissection (CAD) is characterized by an intramural haematoma due to a subintimal tear and accounts for up to 25% of ischaemic strokes in young and middle-aged adults. Data regarding intravenous thrombolysis and endovascular thrombectomy in CAD are scarce and observational – both are reasonably safe and probably recommended. Based on observational evidence, antithrombotic therapy is used to prevent first or recurrent cerebral ischaaemic events in acute or subacute CAD, and event rates are low with either antiplatelet or anticoagulant therapy. The long-term rate of recurrent cerebral ischaemic events or bleeding complications in CAD patients is small while under antithrombotic treatment. Cerebral vasculitis treatment is based on observational series. When primary angiitis of the central nervous system is confirmed by biopsy, a combination of glucocorticoids and cyclophosphamide should be started. Rituximab may be used in patients who are intolerant of cyclophosphamide. In atypical, non-biopsy-proven cases, treatment should be adapted to the severity of neurological involvement. For giant cell arteritis, initial high-dose prednisolone is recommended, beginning a slow taper after 2–4 weeks and continuing at a low dose for 1–2 years. Treatment of p-ANCA-positive and -negative systemic vasculitis with cerebral involvement includes induction corticosteroid therapy followed by addition of cyclophosphamide or other glucocorticoid-sparing drugs.

Keywords

Dissectioncarotid dissectionantiplatelet therapyanticoagulationaspirincerebral vasculitisprimary angiitis of the central nervous systemgiant cell arteritissteroidscyclophosphamidetreatment
Type
Chapter
Information
Stroke Prevention and Treatment
An Evidence-based Approach
 , pp. 450 - 462
Publisher: Cambridge University Press
Print publication year: 2020

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Alrawi, A, Trobe, JD, Blaivas, M, Musch, DC. (1999). Brain biopsy in primary angiitis of the central nervous system. Neurology, 53(4), 858–60.CrossRefGoogle ScholarPubMed
Bejot, Y, Daubail, B, Debette, S, Durier, J, Giroud, M. (2014). Incidence and outcome of cerebrovascular events related to cervical artery dissection: the Dijon Stroke Registry. Int J Stroke, 9(7), 879–82. doi:10.1111/ijs.12154.CrossRefGoogle ScholarPubMed
Berkhemer, OA, Fransen, PS, Beumer, D, van den Berg, LA, Lingsma, HF, Yoo, AJ, et al; MR CLEAN Investigators. (2015). A randomized trial of intraarterial treatment for acute ischemic stroke. N Engl J Med, 372(1), 11–20. doi:10.1056/NEJMoa1411587.CrossRefGoogle ScholarPubMed
CADISS Trial Investigators, Markus, HS, Hayter, E, Levi, C, Feldman, A, Venables, G, Norris, J. (2015). Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial. Lancet Neurol, 14(4), 361–7. doi:10.1016/S1474-4422(15)70018-9.Google Scholar
Campbell, BC, Mitchell, PJ, Kleinig, TJ, Dewey, HM, Churilov, L, Yassi, N, et al., for the EXTEND-IA Investigators. (2015). Endovascular therapy for ischemic stroke with perfusion-imaging selection. N Engl J Med, 372(11), 1009–18. doi:10.1056/NEJMoa1414792.CrossRefGoogle ScholarPubMed
Chowdhury, MM, Sabbagh, CN, Jackson, D, Coughlin, PA, Ghosh, J. (2015). Antithrombotic treatment for acute extracranial carotid artery dissections: a meta-analysis. Eur J Vasc Endovasc Surg, 50(2), 148–56. doi:10.1016/j.ejvs.2015.04.034.Google Scholar
de Boysson, H, Arquizan, C, Guillevin, L, Pagnoux, C. (2013a). Rituximab for primary angiitis of the central nervous system: report of 2 patients from the French COVAC cohort and review of the literature. J Rheumatol, 40(12), 2102–03. doi:10.3899/jrheum.130529CrossRefGoogle ScholarPubMed
de Boysson, H, Boutemy, J, Creveuil, C, Ollivier, Y, Letellier, P, Pagnoux, C, et al. (2013b). Is there a place for cyclophosphamide in the treatment of giant-cell arteritis? A case series and systematic review. Semin Arthritis Rheum, 43(1), 105–12. doi:10.1016/j.semarthrit.2012.12.023.CrossRefGoogle Scholar
de Boysson, H, Zuber, M, Naggara, O, Neau, JP, Gray, F, Bousser, MG, et al; French Vasculitis Study Group and the French NeuroVascular Society. (2014). Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol, 66(5), 1315–26. doi:10.1002/art.38340.Google Scholar
Debette, S, Compter, A, Labeyrie, MA, Uyttenboogaart, M, Metso, TM, Majersik, JJ, et al. (2015). Epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection. Lancet Neurol, 14(6), 640–54. doi:10.1016/S1474-4422(15)00009-5CrossRefGoogle ScholarPubMed
Debette, S, Grond-Ginsbach, C, Bodenant, M, Kloss, M, Engelter, S, Metso, T, et al.; Cervical Artery Dissection Ischemic Stroke Patients Group. (2011a). Differential features of carotid and vertebral artery dissections: the CADISP study. Neurology, 77(12), 1174–81. doi:10.1212/WNL.0b013e31822f03fc.Google Scholar
Debette, S, Leys, D. (2009). Cervical-artery dissections: predisposing factors, diagnosis, and outcome. Lancet Neurol, 8(7), 668–78. doi:10.1016/S1474-4422(09)70084-5.Google Scholar
Debette, S, Metso, T, Pezzini, A, Abboud, S, Metso, A, Leys, D, et al.; Ischemic Stroke Patients Group. (2011b). Association of vascular risk factors with cervical artery dissection and ischemic stroke in young adults. Circulation, 123(14), 1537–44. doi:10.1161/CIRCULATIONAHA.110.000125.CrossRefGoogle ScholarPubMed
Demaerschalk, BM, Kleindorfer, DO, Adeoye, OM, Demchuk, AM, Fugate, JE, Grotta, JC, et al.; American Heart Association Stroke Council and Council on Epidemiology and Prevention. (2016). Scientific rationale for the inclusion and exclusion criteria for intravenous alteplase in acute ischemic stroke: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke, 47(2), 581–641. doi:10.1161/STR.0000000000000086.CrossRefGoogle ScholarPubMed
Emberson, J, Lees, KR, Lyden, P, Blackwell, L, Albers, G, Bluhmki, E, et al.; Stroke Thrombolysis Trialists’ Collaborative Group (2014). Effect of treatment delay, age, and stroke severity on the effects of intravenous thrombolysis with alteplase for acute ischaemic stroke: a meta-analysis of individual patient data from randomised trials. Lancet, 384(9958), 1929–35. doi:10.1016/S0140-6736(14)60584-5.Google Scholar
Engelter, ST, Brandt, T, Debette, S, Caso, V, Lichy, C, Pezzini, A, et al.; Cervical Artery Dissection in Ischemic Stroke Patients Study Group. (2007). Antiplatelets versus anticoagulation in cervical artery dissection. Stroke, 38(9), 2605–11. doi:10.1161/STROKEAHA.107.489666.CrossRefGoogle ScholarPubMed
Engelter, ST, Grond-Ginsbach, C, Metso, TM, Metso, AJ, Kloss, M, Debette, S, et al.; Ischemic Stroke Patients Study, G. (2013). Cervical artery dissection: trauma and other potential mechanical trigger events. Neurology, 80(21), 1950–7. doi:10.1212/WNL.0b013e318293e2eb.Google Scholar
Engelter, ST, Rutgers, MP, Hatz, F, Georgiadis, D, Fluri, F, Sekoranja, L, et al. (2009). Intravenous thrombolysis in stroke attributable to cervical artery dissection. Stroke, 40(12), 3772–6. doi:10.1161/STROKEAHA.109.555953.CrossRefGoogle ScholarPubMed
Engelter, ST, Traenka, C, Lyrer, P. (2017). Dissection of cervical and cerebral arteries. Curr Neurol Neurosci Rep, 17(8), 59. doi:10.1007/s11910-017-0769-3.CrossRefGoogle ScholarPubMed
Falk, RJ, Gross, WL, Guillevin, L, Hoffman, GS, Jayne, DR, Jennette, JC, et al.; American College of Rheumatology; American Society of Nephrology; European League Against Rheumatism. (2011). Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum, 63(4), 863–4. doi:10.1002/art.30286.CrossRefGoogle ScholarPubMed
Ferro, JM. (1998). Vasculitis of the central nervous system. J Neurol, 245(12), 766–76.CrossRefGoogle ScholarPubMed
Gensicke, H, Ahlhelm, F, Jung, S, von Hessling, A, Traenka, C, Goeggel Simonetti, B, et al. (2015). New ischaemic brain lesions in cervical artery dissection stratified to antiplatelets or anticoagulants. Eur J Neurol, 22(5), 859–65, e861. doi:10.1111/ene.12682.Google Scholar
Goyal, M, Demchuk, AM, Menon, BK, Eesa, M, Rempel, JL, Thornton, J, et al.; ESCAPE Trial Investigators. (2015). Randomized assessment of rapid endovascular treatment of ischemic stroke. N Engl J Med, 372(11), 1019–30. doi:10.1056/NEJMoa1414905.Google Scholar
Grau, AJ, Brandt, T, Buggle, F, Orberk, E, Mytilineos, J, Werle, E, et al. (1999). Association of cervical artery dissection with recent infection. Arch Neurol, 56(7), 851–6.Google Scholar
Grond-Ginsbach, C, Debette, S. (2009). The association of connective tissue disorders with cervical artery dissections. Curr Mol Med, 9(2), 210–14.CrossRefGoogle ScholarPubMed
Guellec, D, Cornec-Le Gall, E, Groh, M, Hachulla, E, Karras, A, Charles, P, et al., the French Vasculitis Study Group. (2015). ANCA-associated vasculitis in patients with primary Sjogren’s syndrome: detailed analysis of 7 new cases and systematic literature review. Autoimmun Rev, 14(8), 742–750. doi:10.1016/j.autrev.2015.04.009CrossRefGoogle ScholarPubMed
Guillevin, L, Pagnoux, C. (2003). When should immunosuppressants be prescribed to treat systemic vasculitides? Intern Med, 42(4), 313–17.Google Scholar
Hayreh, SS, Zimmerman, B, Kardon, RH. (2002). Visual improvement with corticosteroid therapy in giant cell arteritis. Report of a large study and review of literature. Acta Ophthalmol Scand, 80(4), 355–67.CrossRefGoogle ScholarPubMed
Hoffman, GS, Cid, MC, Hellmann, DB, Guillevin, L, Stone, JH, Schousboe, J, et al.; International Network for the Study of Systemic Vasculitides (INSSYS). (2002). A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum, 46(5), 1309–18. doi:10.1002/art.10262.CrossRefGoogle ScholarPubMed
Jauch, EC, Saver, JL, Adams, HP Jr, Bruno, A, Connors, JJ, Demaerschalk, BM, et al.; (2013). Guidelines for the early management of patients with acute ischemic stroke: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke, 44(3), 870–947. doi:10.1161/STR.0b013e318284056a.CrossRefGoogle ScholarPubMed
Jennette, JC, Falk, RJ, Milling, DM. (1994). Pathogenesis of vasculitis. Semin Neurol, 14(4), 291–9. doi:10.1055/s-2008-1041088.CrossRefGoogle ScholarPubMed
Jensen, J, Salottolo, K, Frei, D, Loy, D, McCarthy, K, Wagner, J, et al. (2017). Comprehensive analysis of intra-arterial treatment for acute ischemic stroke due to cervical artery dissection. J Neurointerv Surg, 9(7), 654–8. doi:10.1136/neurintsurg-2016-012421.Google Scholar
Jovin, TG, Chamorro, A, Cobo, E, de Miquel, MA, Molina, CA, Rovira, A, et al.; REVASCAT Trial Investigators. (2015). Thrombectomy within 8 hours after symptom onset in ischemic stroke. N Engl J Med, 372(24), 2296–2306. doi:10.1056/NEJMoa1503780.CrossRefGoogle ScholarPubMed
Kennedy, F, Lanfranconi, S, Hicks, C, Reid, J, Gompertz, P, Price, C, et al.; CADISS Investigators. (2012). Antiplatelets vs anticoagulation for dissection: CADISS nonrandomized arm and meta-analysis. Neurology, 79(7), 686–9. doi:10.1212/WNL.0b013e318264e36b.Google Scholar
Kloss, M, Metso, A, Pezzini, A, Leys, D, Giroud, M, Metso, TM, et al. (2012). Towards understanding seasonal variability in cervical artery dissection (CeAD). J Neurol, 259(8), 1662–7. doi:10.1007/s00415-011-6395-0.Google Scholar
Leys, D, Bandu, L, Henon, H, Lucas, C, Mounier-Vehier, F, Rondepierre, P, et al. (2002). Clinical outcome in 287 consecutive young adults (15 to 45 years) with ischemic stroke. Neurology, 59(1), 26–33.Google Scholar
Lin, J, Sun, Y, Zhao, S, Xu, J, Zhao, C. (2016). Safety and efficacy of thrombolysis in cervical artery dissection-related ischemic stroke: a meta-analysis of observational studies. Cerebrovasc Dis, 42(3–4), 272–9. doi:10.1159/000446004.Google Scholar
Lyrer, P, Engelter, S. (2010). Antithrombotic drugs for carotid artery dissection. Cochrane Database Syst Rev, 10. CD000255. doi:10.1002/14651858.CD000255.pub2.Google Scholar
Marnat, G, Mourand, I, Eker, O, Machi, P, Arquizan, C, Riquelme, C, et al. (2016). Endovascular management of tandem occlusion stroke related to internal carotid artery dissection using a distal to proximal approach: insight from the RECOST Study. AJNR Am J Neuroradiol, 37(7), 1281–8. doi:10.3174/ajnr.A4752.CrossRefGoogle ScholarPubMed
Mazlumzadeh, M, Hunder, GG, Easley, KA, Calamia, KT, Matteson, EL, Griffing, WL, et al. (2006). Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial. Arthritis Rheum, 54(10), 3310–18. doi:10.1002/art.22163.Google Scholar
Menon, R, Kerry, S, Norris, JW, Markus, HS. (2008). Treatment of cervical artery dissection: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry, 79(10), 1122–7. doi:10.1136/jnnp.2007.138800Google Scholar
Metso, AJ, Metso, TM, Debette, S, Dallongeville, J, Lyrer, PA, Pezzini, A, et al. (2012). Gender and cervical artery dissection. Eur J Neurol, 19(4), 594–602. doi:10.1111/j.1468-1331.2011.03586.x.Google Scholar
Metso, TM, Tatlisumak, T, Debette, S, Dallongeville, J, Engelter, ST, Lyrer, PA, et al. (2012). Migraine in cervical artery dissection and ischemic stroke patients. Neurology, 78(16), 1221–8. doi:10.1212/WNL.0b013e318251595fGoogle Scholar
Moosig, F, Bremer, JP, Hellmich, B, Holle, JU, Holl-Ulrich, K, Laudien, M, et al. (2013). A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis, 72(6), 1011–17. doi:10.1136/annrheumdis-2012-201531.Google Scholar
Mouthon, L, Dunogue, B, Guillevin, L. (2014). Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun, 48–49, 99–103. doi:10.1016/j.jaut.2014.01.018Google Scholar
Myles, AB. (1992). Steroid treatment in giant cell arteritis. Br J Rheumatol, 31(11), 787.Google Scholar
Nebelsieck, J, Sengelhoff, C, Nassenstein, I, Maintz, D, Kuhlenbaumer, G, Nabavi, DG, et al. (2009). Sensitivity of neurovascular ultrasound for the detection of spontaneous cervical artery dissection. J Clin Neurosci, 16(1), 79–82. doi:10.1016/j.jocn.2008.04.005.Google Scholar
Nedeltchev, K, der Maur, TA, Georgiadis, D, Arnold, M, Caso, V, Mattle, HP, et al. (2005). Ischaemic stroke in young adults: predictors of outcome and recurrence. J Neurol Neurosurg Psychiatry, 76(2), 191–5. doi:10.1136/jnnp.2004.040543CrossRefGoogle ScholarPubMed
Puppo, C, Massollo, M, Paparo, F, Camellino, D, Piccardo, A, Shoushtari Zadeh Naseri, M, et al. (2014). Giant cell arteritis: a systematic review of the qualitative and semiquantitative methods to assess vasculitis with 18 F-fluorodeoxyglucose positron emission tomography. Biomed Res Int, 2014, 574248. doi:10.1155/2014/574248.Google Scholar
Ruegg, S, Engelter, S, Jeanneret, C, Hetzel, A, Probst, A, Steck, AJ, Lyrer, P. (2003). Bilateral vertebral artery occlusion resulting from giant cell arteritis: report of 3 cases and review of the literature. Medicine (Baltimore), 82(1), 1–12.Google Scholar
Salvarani, C, Brown, RD, Jr, Calamia, KT, Christianson, TJ, Weigand, SD, Miller, DV, et al. (2007). Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol, 62(5), 442–51. doi:10.1002/ana.21226.Google Scholar
Salvarani, C, Brown, RD Jr, Christianson, T, Miller, DV, Giannini, C, Huston, J 3rd, et al. (2015). An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients. Medicine (Baltimore), 94(21), e738. doi:10.1097/MD.0000000000000738Google Scholar
Salvarani, C, Brown, RD, Jr, Hunder, GG. (2017). Adult primary central nervous system vasculitis. Isr Med Assoc J, 19(7), 448–53.Google ScholarPubMed
Salvarani, C, Brown, RD, Jr., Huston, J, 3rd, Morris, J M, Hunder, GG. (2014). Treatment of primary CNS vasculitis with rituximab: case report. Neurology, 82(14), 1287–8. doi:10.1212/WNL.0000000000000293.Google Scholar
Salvarani, C, Cantini, F, Boiardi, L, Hunder, GG. (2002). Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med, 347(4), 261–71. doi:10.1056/NEJMra011913.Google Scholar
Salvarani, C, Hatemi, G. (2019). Management of large-vessel vasculitis. Curr Opin Rheumatol, 31(1), 25–31. doi:10.1097/BOR.0000000000000561.Google Scholar
Sarikaya, H, da Costa, BR, Baumgartner, RW, Duclos, K, Touze, E, de Bray, JM, et al. (2013). Antiplatelets versus anticoagulants for the treatment of cervical artery dissection: Bayesian meta-analysis. PLoS One, 8(9), e72697. doi:10.1371/journal.pone.0072697.Google Scholar
Saver, JL, Goyal, M, Bonafe, A, Diener, HC, Levy, EI, Pereira, VM, et al.; SWIFT PRIME Investigators. (2015). Stent-retriever thrombectomy after intravenous t-PA vs. t-PA alone in stroke. N Engl J Med, 372(24), 2285–95. doi:10.1056/NEJMoa1415061.Google Scholar
Schievink, WI. (2001). Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med, 344(12), 898–906. doi:10.1056/NEJM200103223441206.Google Scholar
Schievink, WI, Mokri, B, Whisnant, JP. (1993). Internal carotid artery dissection in a community. Rochester, Minnesota, 1987–1992. Stroke, 24(11), 1678–80.Google Scholar
Siva, A. (2001). Vasculitis of the nervous system. J Neurol, 248(6), 451–68.Google Scholar
Stone, JH, Tuckwell, K, Dimonaco, S, Klearman, M, Aringer, M, Blockmans, D, et al. (2017). Trial of Tocilizumab in Giant-Cell Arteritis. N Engl J Med, 377(4), 317–28. doi:10.1056/NEJMoa1613849.Google Scholar
Torres, J, Loomis, C, Cucchiara, B, Smith, M, Messe, S. (2016). Diagnostic yield and safety of brain biopsy for suspected primary central nervous system angiitis. Stroke, 47(8), 2127–9. doi:10.1161/STROKEAHA.116.013874.Google Scholar
Traenka, C, Jung, S, Gralla, J, Kurmann, R, Stippich, C, Simonetti, BG, et al. (2018). Endovascular therapy versus intravenous thrombolysis in cervical artery dissection ischemic stroke – results from the SWISS registry. Eur Stroke J, 3(1), 47–56. doi:10.1177/2396987317748545.Google Scholar
Unizony, S, Arias-Urdaneta, L, Miloslavsky, E, Arvikar, S, Khosroshahi, A, Keroack, B, et al. (2012). Tocilizumab for the treatment of large-vessel vasculitis (giant cell arteritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res (Hoboken), 64(11), 1720–9. doi:10.1002/acr.21750.Google Scholar
Wardlaw, JM, Murray, V, Berge, E, del Zoppo, GJ. (2014). Thrombolysis for acute ischaemic stroke. Cochrane Database Syst Rev, 7. CD000213. doi:10.1002/14651858.CD000213.pub3.Google ScholarPubMed
Zinkstok, SM, Vergouwen, MD, Engelter, ST, Lyrer, PA, Bonati, LH, Arnold, M, et al. (2011). Safety and functional outcome of thrombolysis in dissection-related ischemic stroke: a meta-analysis of individual patient data. Stroke, 42(9), 2515–20. doi:10.1161/STROKEAHA.111.617282.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×