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Central Nervous System Vasculitis: Immunopathogenesis, Clinical Aspects, and Treatment Options

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Inflammatory Disorders of the Nervous System

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

Inflammation is a naturally occurring phenomenon in stroke and can be either a nonspecific reaction to a vascular insult or a primary component of the pathogenesis. The potential for the inflammation associated with connective tissue disorders to affect the cerebral or spinal circulation is well recognized. Perhaps the most common vasculitic process of the cerebral circulation encountered is temporal, i.e., giant cell arteritis. Determination of a primary component of vascular inflammation in the patient’s presentation can be quite challenging and remains extremely important as it will be vital in determining whether or not immunosuppressive therapy is indicated. Documentation of the vasculitic process in a timely fashion, the need for potent immunosuppressive therapy, as well as the choice and dosing of therapy, along with the duration, are important determinants of outcome in central nervous vasculitis. We hope to provide updated information toward these goals in this chapter.

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References

  1. Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol. 2011;10:561–72.

    Article  PubMed  Google Scholar 

  2. Birnbaum J, Hellman DB. Primary angiitis of the central nervous system. Arch Neurol. 2009;66:704–9.

    Article  PubMed  Google Scholar 

  3. Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum. 1997;40:1189–201.

    Article  CAS  PubMed  Google Scholar 

  4. Salvarani C, Brown Jr RD, Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007;62:442–51.

    Article  PubMed  Google Scholar 

  5. Lie JT. Angiitis of the central nervous system. Curr Opin Rheumatol. 1991;3:36–45.

    Article  CAS  PubMed  Google Scholar 

  6. Salvarani C, Brown Jr RD, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement. Neurology. 2008;70:2394–400.

    Article  CAS  PubMed  Google Scholar 

  7. Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OA. Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges. Arch Neurol. 1993;50:925–30.

    Article  CAS  PubMed  Google Scholar 

  8. Parisi JE, Moore PM. The role of biopsy in vasculitis of the central nervous system. Semin Neurol. 1994;14:341–8.

    Article  CAS  PubMed  Google Scholar 

  9. Salvarani C, Brown Jr RD, Calamia KT, et al. Primary central nervous system vasculitis with prominent leptomeningeal enhancement: a subset with a benign outcome. Arthritis Rheum. 2008;58:595–603.

    Article  PubMed  Google Scholar 

  10. Salvarani C, Brown Jr RD, Christianson T, et al. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis. Description of 163 patients. Medicine. 2015;21:1–15.

    Google Scholar 

  11. Weyand CM, Goronzy JJ. Giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014;4(371):50–7.

    Google Scholar 

  12. Broussalis E, Trinka E, Draus J, McCoy M, Killer M. Treatment strategies for vasculitis that affects the nervous system. Drug Discov Today. 2013;18:818–35.

    Article  PubMed  Google Scholar 

  13. Berlit P. Diagnosis and treatment of cerebral vasculitis. Ther Adv Neurol Disord. 2010;3(3):29–42.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–98.

    Article  CAS  PubMed  Google Scholar 

  15. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310–7.

    Article  CAS  PubMed  Google Scholar 

  16. Salvarani C, Brown Jr RD, Hunder GG. Adult primary central nervous system vasculitis. Lancet. 2012;380:767–77.

    Article  PubMed  Google Scholar 

  17. Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke. 1988;19:1159–70.

    Article  CAS  PubMed  Google Scholar 

  18. Sendara M, Chiras J, Cujas M, Lhermitte F. Isolated benign cerebral vasculitis or migrainous vasospasm? J Neurol Neurosurg Psychiatry. 1984;47:73–6.

    Article  Google Scholar 

  19. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible vasoconstriction syndromes. Ann Intern Med. 2007;146:34–44.

    Article  PubMed  Google Scholar 

  20. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol. 2009;9:256–67.

    Article  PubMed  Google Scholar 

  21. Wang SJ, Fuh JL, Wu JA, Chen SP, Lai TH. Bath-related thunderclap headache: a study of 21 consecutive patients. Cephalalgia. 2008;28:524–30.

    Article  PubMed  Google Scholar 

  22. Hu CM, Lin YJ, Fan YK, Chen SP, Lai TH. Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci. 2010;17:1349–51.

    Article  PubMed  Google Scholar 

  23. Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11:906–17.

    Article  PubMed  Google Scholar 

  24. Ducros A, Boukobaza M, Porcher R, Sarow M, Valade D, Bousser MG. Brain. 2007;130:3091–101.

    Article  PubMed  Google Scholar 

  25. Edlow JA, Caplan LR, O’Brien K, Tibbles CD. Diagnosis of acute neurlological emergencies in pregnant and post-partum women. Lancet Neurol. 2013;12:175–85.

    Article  PubMed  Google Scholar 

  26. Fugate JE, Ameriso SF, Ortiz G, et al. Variable presentations of postpartum angiopathy. Stroke. 2012;43:67–676.

    Article  Google Scholar 

  27. Bartynski WS. Posterior reversible encephalopathic syndrome. Part 1: fundamental neuroimaging and clinical features. AJNR Am J Neuroradiol. 2008;29:1036–42.

    Article  CAS  PubMed  Google Scholar 

  28. Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc. 2010;85:427–32.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Ibrahim NMA, Badawy ME. MR imaging of posterior reversible encephalopathy syndrome associated with pregnancy. Egypt J Radiol Nucl Med. 2014;45:505–10.

    Article  Google Scholar 

  30. Staykov D, Schwab S. Posterior reversible encephalopathy syndrome. J Intensive Care. 2012;27:11–24.

    Article  Google Scholar 

  31. Jackson M, Lennox G, Jaspan T, Jefferson D. Migrainous angiitis precipitated by sex Headache and leading to watershed infarction. Cephalalgia. 1993;13:427–30.

    Article  CAS  PubMed  Google Scholar 

  32. Serdaru M, Chiras J, Cujas M, Lhermitte F. Isolated benign cerebral vasculitis or migrainous vasospasm? J Neurol Neurosurg Psychiatry. 1984;47:73–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Singhal AB, Caviness VS, Begleiter AF, Mark EJ, Rordorf G, Korosshetz WJ. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology. 2002;58:130–3.

    Article  CAS  PubMed  Google Scholar 

  34. Meschia JF, Malkoff MD, Biller J. Reversible segmental cerebral arterial vasospasm and cerebral infarction: possible association with excessive use of sumatriptan and Midrin. Arch Neurol. 1998;55:712–4.

    Article  CAS  PubMed  Google Scholar 

  35. Ducros A, Fiedler U, Porcher R, Boukobza M, Stapf C, Bousser MG. Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: frequency, features and risk factors. Stroke. 2010;41:2505–11.

    Article  PubMed  Google Scholar 

  36. Gonzalez-Gay MA, Vazquez-Rodriquez TR, Lopez-Diaz MJ, et al. Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum. 2009;61:1454–61.

    Article  PubMed  Google Scholar 

  37. Chacko JG, Chacko JA, Salter MW. Review of giant cell arteritis. Saudi J Ophthalmol. 2015;29:48–52.

    Article  PubMed  Google Scholar 

  38. Hunder GG, Da B, Michel BA, et al. The American College of Rheumatology 1990 criteria for classification of giant cell arteritis. Arthritis Rheum. 1990;33:1122–8.

    Article  CAS  PubMed  Google Scholar 

  39. Ly K-H, Regent MC, Mouthon L. Pathogenesis of giant cell arteritis: more than just an inflammatory condition? Autoimmun Rev. 2010;9:635–45.

    Article  CAS  PubMed  Google Scholar 

  40. Weyand CM, Goronzy JJ. Giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014;371:50–7.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  41. Weyand CM, Goronzy JJ. Immune mechanisms in medium and large-vessel vasculitis. Nat Rev Rheumatol. 2013;9:731–40.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Gilden D, Nagel M. Varicella zoster virus in temporal arteries of patients with giant cell arteritis. J Infect Dis. 2015;212(Supple 1):S37–9.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Gilden D, White T, Khmeleva N, et al. Prevalence and distribution of VZV in temporal arteries of patients with giant cell arteritis. Neurology. 2015;84:1948–55.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Aiello PD, Trautmann JC, McPhee TJ, Kunselman AR, Hunder GG. Visual prognosis in giant cell arteritis. Ophthalmology. 1993;100:550–5.

    Article  CAS  PubMed  Google Scholar 

  45. Azhar SS, Tang RA, Dorotheo EU. Giant cell arteritis: diagnosing and treating inflammatory disorders in older adults. Geriatrics. 2005;60:26–30.

    PubMed  Google Scholar 

  46. Guida A, Tufano A, Perna P, et al. The thromboembolic risk in Giant Cell Arteritis: a critical review of the literature. Int J Rheumatol. 2014;1:1–7.

    Article  Google Scholar 

  47. Murchison AP, Gilbert ME, Bikyk JR, et al. Validity of the American College of Rheumatology Criteria for the diagnosis of giant cell arteritis. Am J Ophthalmol. 2012;154:617–9.

    Article  Google Scholar 

  48. Kermani TA, Schmidt J, Crowson CS, et al. Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of temporal arteritis. Semin Arthritis Rheum. 2012;41:866–71.

    Article  CAS  PubMed  Google Scholar 

  49. Kermani TA, Warrington KJ, Crowson CS, et al. Large-vessel involvement in giant cell arteritis: a population-based cohort study of the incidence and prognosis. Ann Rheum Dis. 2013;72:1989–94.

    Article  PubMed  Google Scholar 

  50. Saedon H, Saedon M, Goodyear S, Papettas T, Marshall C. Temporal artery biopsy for giant cell arteritis. Retrospective audit. JRSM Short Rep. 2012;3:73.

    Article  PubMed  PubMed Central  Google Scholar 

  51. Kotter I, Henes JC, Wagner AD, Loock J, Gross WL. Does glucocorticosteroid-resistant Large-vessel vasculitis (giant cell arteritis and Takayasu arteritis) exist and how can remission be achieved? A critical review of the literature. Clin Exp Rheumatol. 2012;30(Suppl 70):S114–29.

    PubMed  Google Scholar 

  52. Samson M, Puechal X, Devilliers H, et al. Long-term follow-up of a randomized trial on 118 patients with polyarteritis nodosa or microscopic polyangiitis without poor-prognosis factors. Autoimmun Rev. 2014;13:197–205.

    Article  PubMed  Google Scholar 

  53. Younger DS. Vasculitis of the nervous system. Curr Opin Neurol. 2004;17:317–36.

    Article  PubMed  Google Scholar 

  54. Bougea A, Anagnostou E, Spandideas N, Triantafyllou N, Kararizou E. An Update of neurological manifestations of vasculitides and connective tissue diseases: a literature review. Einstein (Open Access). 2015;13:627–35.

    Article  Google Scholar 

  55. Reichart MD, Bogousslavsky J, Janzer RC. Early lacunar strokes complicating polyarteritis nodosa: thrombotic microangiopathy. Neurology. 2000;54:883–9.

    Article  CAS  PubMed  Google Scholar 

  56. Vishwanath S, Relan M, Shen L, Ambrus Jr JL. Update on the use of biologics in vasculitides. Curr Pharm Biotechnol. 2014;15:558–62.

    Article  CAS  PubMed  Google Scholar 

  57. Ribi C, Cohen P, Pagnous C, et al. French Vasculitis Study Group. Treatment of polyarteritis nodosa and microscopic polyangiitis without poor prognosis factors. A prospective randomized study of one hundred twenty-four patients. Arthritis Rheum. 2010;62:1186–1197.

    Google Scholar 

  58. Pagnoux C, Seror R, Henegar C, et al. for the French Vasculitis Study Group. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group database. Arthritis Rheum. 2010;62:616–626.

    Google Scholar 

  59. Semmo AN, Baumert TF, Kreisel W. Severe cerebral vasculitis as primary manifestation of hepatitis-B-associated polyarteritis nodosa. J Hepatol. 2002;37:414–6.

    Article  PubMed  Google Scholar 

  60. Salvarani C, Pipitone N, Hunder GG. Management of primary and secondary central nervous system vasculitis. Curr Opin Rheumatol. 2016;28:21–28.

    Google Scholar 

  61. Seror R, Mahr A, Ramanoelina J, et al. Central nervous system involvement in Wegener’s granulomatosis. Medicine. 2006;85:54–65.

    Article  PubMed  Google Scholar 

  62. Holle JU, Gross WL. Neurological involvement in Wegener’s granulomatosis. Curr Opin Rheumatol. 2011;23:7–11.

    Article  PubMed  Google Scholar 

  63. Fadil H, Gonzalez-Toledo E, Kelley RE, Henley J. Wegener’s granulomatosis with extension to the cavernous sinus. J La State Med Soc. 2007;159:212–4.

    PubMed  Google Scholar 

  64. Nishino H, Rubino FA, DeRemee RA, et al. Neurological involvement Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33:4–9.

    Article  CAS  PubMed  Google Scholar 

  65. Ku BD, Shin HY. Multiple bilateral non-hemorrhagic cerebral infarctions associated with microscopic polyangiitis. Clin Neurol Neurosurg. 2009;111:904–6.

    Article  PubMed  Google Scholar 

  66. Ozkul A, Tataroglu C, Kiylioglu N, Akoyi A, Tataroglu C. Microscopic polyangiitis presenting with medullary infarct. J Neurol Sci. 2011;300:173–5.

    Article  PubMed  Google Scholar 

  67. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094–100.

    Article  CAS  PubMed  Google Scholar 

  68. Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ. Neurological complications of Churg-Strauss syndrome-a prospective monocentric study. Eur J Neurol. 2010;17:582–8.

    Article  CAS  PubMed  Google Scholar 

  69. Ribi C, Cohen P, Pagnoux C, et al. Treatment of Churg-Strauss syndrome without poor- prognosis factors: a multicenter, prospective, randomized open-label study of seventy-two patients. Arthritis Rheum. 2008;58:586–94.

    Article  CAS  PubMed  Google Scholar 

  70. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA- associated vasculitis. N Engl J Med. 2010;363:221–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  71. Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA- associated renal vasculitis. N Engl J Med. 2010;363:211–20.

    Article  CAS  PubMed  Google Scholar 

  72. Pagnoux C, Mahr A, Hamidou MA, et al. Azothrioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790–803.

    Article  CAS  PubMed  Google Scholar 

  73. Kashiwagi T, Hayama N, Fujita E, et al. A case of (double) negative granulomatosis with polyangiitis (Wegener’s). CEN Case Rep. 2012;1:104–11.

    Article  Google Scholar 

  74. Guellec D, Cornec-Le Gall E, Groh M, et al. ANCA-associated vasculitis in patients with primary Sjogren’s syndrome: detailed analysis of 7 new cases and systemic review of the literature. Autoimmun Rev. 2015;14:742–50.

    Article  CAS  PubMed  Google Scholar 

  75. Subramanyan R, Joy J, Balakrishman KG. Natural history of aortoarteritis (Takayasu’s disease). Circulation. 1989;80:429–37.

    Article  CAS  PubMed  Google Scholar 

  76. Wen D, Du X, Ma CS. Takayasu arteritis: diagnosis, treatment and prognosis. Int Rev Immunol. 2012;31:462–73.

    Article  CAS  PubMed  Google Scholar 

  77. Hirohata S. Central nervous system involvement in Behcet’s disease. Rinsho Shinkeigaku. 2001;41:1147–9.

    CAS  PubMed  Google Scholar 

  78. Kidd D. Neurological complications of Behcet’s syndrome. Curr Neurol Neurosci Rep. 2012;12:675–9.

    Article  CAS  PubMed  Google Scholar 

  79. Saip S, Akman-Demir G, Siva A. Neuro-Behcet syndrome. Handb Clin Neurol. 2014;121:1703–23.

    Article  PubMed  Google Scholar 

  80. Kikuchi H, Aramaki K, Hirohata S. Low dose MTX for progressive neuro-Behcet’s disease. A follow-up for 4 years. Adv Exp Med Biol. 2003;528:575–8.

    Article  CAS  PubMed  Google Scholar 

  81. Espinoza GM, Prost A. Cogan’s syndrome and other ocular vasculitides. Curr Rheumatol Rep. 2015;17:24–7.

    Article  PubMed  Google Scholar 

  82. Angiletta D, Wiesel P, Marinazzo D, Bortone AS, Regina G. Endovascular treatment of multiple aneurysms complicating Cogan syndrome. Ann Vasc Surg. 2015;29:361–0.

    Article  PubMed  Google Scholar 

  83. Singer O. Cogan and Behcet syndromes. Rheum Dis Clin North Am. 2015;41:75–9.

    Article  PubMed  Google Scholar 

  84. Antonios N, Silliman S. Cogan syndrome: an analysis of reported neurological manifestations. Neurologist. 2012;18:55–63.

    Article  PubMed  Google Scholar 

  85. Dorr J, Krautwald S, Wildeman B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9:307–16.

    Article  PubMed  Google Scholar 

  86. Kleffner I, Duning T, Lohmann H, et al. A brief review of Susac syndrome. J Neurol Sci. 2012;322:35–40.

    Article  CAS  PubMed  Google Scholar 

  87. Cacoub P, Comarmond C, Domont F, Savey L, Saadoun D. Cryoglobulinemic vasculitis. Am J Med. 2015;128:950–5.

    Article  PubMed  Google Scholar 

  88. Ferr C, Sebastiani M, Colaci M, Poupak F, Piluso A, Antonelli A, Zignego AL. Hepatitis C virus syndrome: a constellation of organ- and non-organ specific disorders, B-cell non-Hodgkins lymphoma, and cancer. World J Hepatol. 2015;7:327–43.

    Article  Google Scholar 

  89. De Vita S, Quartuccio L, Isola M, et al. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum. 2012;64:843–53.

    Article  CAS  PubMed  Google Scholar 

  90. Lally L, Spiera R. B-cell-targeted therapy in systemic vasculitis. Curr Opin Rheumatol. 2016;28:15–20.

    Article  CAS  PubMed  Google Scholar 

  91. Greenberg BM. The neurological manifestations of systemic lupus erythematosus. Neurologist. 2009;15:115–21.

    Article  PubMed  Google Scholar 

  92. Kelley RE, Stokes N, Reyes P, Harik S. Cerebral transmural angiitis and ruptured aneurysm. Arch Neurol. 1980;37:526–7.

    Article  CAS  PubMed  Google Scholar 

  93. Streifler JY, Molad Y. Connective tissue disorders: systemic lupus erythematosus, Sjogren’s syndrome and scleroderma. Handb Clin Neurol. 2014;119:463–73.

    Article  PubMed  Google Scholar 

  94. Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S. Neurologic involvement in Scleroderma: a systematic review. Semin Arthritis Rheum. 2013;43:335–47.

    Article  PubMed  Google Scholar 

  95. de Corte FC, Neves N. Cervical spine instability in rheumatoid arthritis. Eur J Orthop Surg Traumatol. 2014;24(Suppl 1):S83–91.

    PubMed  Google Scholar 

  96. Nogueras C, Sala M, Sasal M, et al. Recurrent stroke as a manifestation of primary angiitis of the central nervous system in a patient infected with human immunodeficiency virus. Arch Neurol. 2002;59:468–73.

    Article  PubMed  Google Scholar 

  97. Garcia-Garcia JA, Macias J, Castellanos V, et al. Necrotizing granulomatous vasculitis in advanced HIV infection. J Infect. 2003;47:333–5.

    Article  PubMed  Google Scholar 

  98. Savige JA, Chang L, Horn S, Crowe SM. Antinuclear, anti-neutrophil cytoplasmic and anti- glomerular basement membrane antibodies in HIV-infected individuals. Autoimmunity. 1994;18:205–11.

    Article  CAS  PubMed  Google Scholar 

  99. McKelvie PA, Collins S, Thyagarajan D, Trost N, Sheorey H, Byrne E. Meningoencephalo-myelitis with vasculitis due to varicella zoster virus: a case report and review of the literature. Pathology. 2002;34:88–93.

    Article  PubMed  Google Scholar 

  100. Gilden DH, Kleinschmidt-DeMasters BK, LaGuardia JJ, Mahalingam R, Cohrs RJ. Neurologic complications of reactivation of varicella-zoster virus. N Engl J Med. 2000;342:635–45.

    Article  CAS  PubMed  Google Scholar 

  101. Grahn A, Studahl M. Varicella-zoster virus infections of the central nervous system- Prognosis, diagnostics and treatment. J Infect. 2015;71:281–93.

    Article  PubMed  Google Scholar 

  102. Russman AN, Lederman RJ, Calabrese LH, Embi PJ, Forghani B, Gilden DH. Multifocal Varicella-zoster virus vasculopathy without rash. Arch Neurol. 2003;60:1607–9.

    Article  PubMed  Google Scholar 

  103. Teng GG, Chatham WW. Vasculitis related to viral and other microbial agents. Best Pract Res Clin Rheumatol. 2015;29:226–43.

    Article  PubMed  Google Scholar 

  104. Smadja D, Mas JL, Fallet-Bianco C, Sicard D, de Recondo J, Rondot P. Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review. J Neurooncol. 1991;11:171–80.

    Article  CAS  PubMed  Google Scholar 

  105. Gaul C, Hannisch F, Neureiter D, Behrmann C, Neundorfer B, Winterhoffer M. Intravascular lymphomatosis mimicking disseminated encephalomyelitis and Encephalomyelopathy. Clin Neurol Neurosurg. 2006;108:486–9.

    Article  PubMed  Google Scholar 

  106. Berger JR, Jone R, Wilson D. Intravascular lymphomatosis presenting with sudden hearing loss. J Neurol Sci. 2005;232:105–9.

    Article  PubMed  Google Scholar 

  107. Vieren M, Sciot R, Robberecht W. Intravascular lymphomatosis of the brain: a diagnostic problem. Clin Neurol Neurosurg. 1999;101:33–6.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, Tulane University School of Medicine, 1430 Tulane Avenue 8065, New Orleans, LA, 70112, USA

    Roger E. Kelley MD, Ramy El-Khoury MD & Brian P. Kelley BS

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  1. Roger E. Kelley MD
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  2. Ramy El-Khoury MD
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Correspondence to Roger E. Kelley MD .

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Editors and Affiliations

  1. Department of Neurology, LSU Health Sciences Center, Shreveport, Louisiana, USA

    Alireza Minagar

  2. Department of Molecular and Cellular Physiology, LSU Health Sciences Center, Shreveport, Louisiana, USA

    J. Steven Alexander

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Kelley, R.E., El-Khoury, R., Kelley, B.P. (2017). Central Nervous System Vasculitis: Immunopathogenesis, Clinical Aspects, and Treatment Options. In: Minagar, A., Alexander, J. (eds) Inflammatory Disorders of the Nervous System. Current Clinical Neurology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-51220-4_4

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