Skip to main content
SpringerLink
Log in

Central Nervous System Vasculitis: Primary Angiitis of the Central Nervous System

  • Protocol
  • First Online:
Cerebrovascular Disorders

Part of the book series: Neuromethods ((NM,volume 170))

  • 664 Accesses

Abstract

Primary angiitis of the central nervous system (CNS) is a rare disorder involving idiopathic inflammation of small- and medium-sized leptomeningeal and parenchymal arteries within the CNS. The disease may cause headaches, multifocal strokes of either ischemic or hemorrhagic subtypes, cognitive impairment, seizures, and visual changes; however, there is a wide variation in symptomatology and clinical course of patients presenting with primary angiitis of the CNS. This chapter reviews the standard diagnostic evaluation for primary angiitis of the CNS, which includes serological testing for signs of inflammation and potential alternative diagnoses, vascular neuroimaging, lumbar puncture, diagnostic cerebral angiogram, and possible brain biopsy. Although the disease carries high mortality if left untreated, it is relatively responsive to corticosteroids as part of an acute treatment course. This chapter also reviews several immunosuppressive agents used for maintenance therapy.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Protocol
USD 49.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

ABRA:

Aβ-related angiitis

ACE:

Angiotensin-converting enzyme

ANCA:

Antineutrophil cytoplasmic antibodies

CNS:

Central nervous system

CRP:

C-reactive protein

CSF:

Cerebrospinal fluid

EBV:

Epstein-Barr virus

ESR:

Erythrocyte sedimentation rate

HHV-6:

Human herpesvirus 6

HIV:

Human immunodeficiency virus

HSV:

Herpes simplex virus

MRA:

Magnetic resonance angiography

MRI:

Magnetic resonance imaging

mRS:

Modified Rankin Scale

PACNS:

Primary angiitis of the CNS

PRES:

Posterior reversible encephalopathy syndrome

RCVS:

Reversible cerebral vasoconstriction syndrome

SSA/B:

Sjögren’s syndrome-related antigen A/B

VDRL:

Venereal disease research laboratory test

VZV:

Varicella zoster virus

References

  1. Salvarani C, Brown RD, Christianson T 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:e738. https://doi.org/10.1097/MD.0000000000000738

    Article  CAS  Google Scholar 

  2. Salvarani C, Brown RD, Calamia KT et al (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 62:442–451. https://doi.org/10.1002/ana.21226

    Article  PubMed  Google Scholar 

  3. Arthur G, Margolis G (1977) Mycoplasma-like structures in granulomatous angiitis of the central nervous system. Case reports with light and electron microscopic studies. Arch Pathol Lab Med 101:382–387

    CAS  PubMed  Google Scholar 

  4. Thomas L, Davidson M, McClusky RT (1966) Studies of PPLO infection: the production of cerebral polyarteritis by mycoplasma gallisepticum in turkeys, the neurotoxic properties of the mycoplasma. J Exp Med 123:897–912

    Article  CAS  Google Scholar 

  5. Reyes MG, Fresco R, Chokroverty S, Salud EQ (1976) Viruslike particles in granulomatous angiitis of the central nervous system. Neurology 26:797–799

    Article  CAS  Google Scholar 

  6. Hajj-Ali RA, Calabrese LH (2014) Diagnosis and classification of central nervous system vasculitis. J Autoimmun 48–49:149–152. https://doi.org/10.1016/j.jaut.2014.01.007

    Article  PubMed  Google Scholar 

  7. Salvarani C, Brown RD, Calamia KT et al (2011) Rapidly progressive primary central nervous system vasculitis. Rheumatology (Oxford) 50:349–358. https://doi.org/10.1093/rheumatology/keq303

    Article  Google Scholar 

  8. Scolding NJ, Joseph F, Kirby PA et al (2005) Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 128:500–515. https://doi.org/10.1093/brain/awh379

    Article  PubMed  Google Scholar 

  9. De Boysson H, Zuber M, Naggara O et al (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 66:1315–1326. https://doi.org/10.1002/art.38340

    Article  PubMed  Google Scholar 

  10. Ducros A (2012) Reversible cerebral vasoconstriction syndrome. Lancet Neurol 11:906–917. https://doi.org/10.1016/S1474-4422(12)70135-7

    Article  PubMed  Google Scholar 

  11. Nagel MA, Cohrs RJ, Mahalingham R et al (2018) The varicella zoster vasculopathies: clinical, Csf, imaging and virologic features. Neurology 70:853–860. https://doi.org/10.1212/01.wnl.0000304747.38502.e8.The

    Article  Google Scholar 

  12. Gaa J, Weidauer S, Sitzer M et al (2004) Cerebral vasculitis due to Treponema pallidum infection: MRI and MRA findings [2]. Eur Radiol 14:746–747. https://doi.org/10.1007/s00330-003-2015-4

    Article  PubMed  Google Scholar 

  13. Melica G, Brugieres P, Lascaux A-S et al (2009) Primary vasculitis of the central nervous system in patients infected with HIV-1 in the HAART era. J Med Virol 81:578–581. https://doi.org/10.1002/jmv

    Article  PubMed  Google Scholar 

  14. Katchanov J, Siebert E, Klingebiel R, Endres M (2010) Infectious vasculopathy of intracranial large- and medium-sized vessels in neurological intensive care unit: a clinico-radiological study. Neurocrit Care 12:369–374. https://doi.org/10.1007/s12028-010-9335-4

    Article  CAS  PubMed  Google Scholar 

  15. Al-Araji A, Kidd DP (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8:192–204. https://doi.org/10.1016/S1474-4422(09)70015-8

    Article  PubMed  Google Scholar 

  16. Al-Araji A, Sharquie K, Al-Rawi Z (2003) Prevalence and patterns of neurological involvement in Behcet’s disease: a prospective study from Iraq. J Neurol Neurosurg Psychiatry 74:608–613. https://doi.org/10.1136/jnnp.74.5.608

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Hirohata S (2008) Histopathology of central nervous system lesions in Behcet’s disease. J Neurol Sci 267:41–47. https://doi.org/10.1016/j.jns.2007.09.041

    Article  CAS  PubMed  Google Scholar 

  18. Wludarczyk A, Szczeklik W (2016) Neurological manifestations in ANCA-associated vasculitis – assessment and treatment. Expert Rev Neurother 7175:861–863. https://doi.org/10.1586/14737175.2016.1165095

    Article  CAS  Google Scholar 

  19. Suppiah R, Hadden RDM, Batra R et al (2011) Peripheral neuropathy in ANCA-associated vasculitis: outcomes from the European Vasculitis Study Group trials. Rheumatology 50:2214–2222. https://doi.org/10.1093/rheumatology/ker266

    Article  PubMed  Google Scholar 

  20. Gwathmey KG, Burns TM, Collins MP, Dyck PJB (2014) Vasculitic neuropathies. Lancet Neurol 13:67–82. https://doi.org/10.1016/S1474-4422(13)70236-9

    Article  PubMed  Google Scholar 

  21. Salvarani C, Brown RD, Hunder GG (2012) Adult primary central nervous system vasculitis. Lancet 380:767–777. https://doi.org/10.1016/S0140-6736(12)60069-5

    Article  PubMed  Google Scholar 

  22. Salvarani C, Giannini C, Miller DV, Hunder G (2006) Giant cell arteritis: involvement of intracranial arteries. Arthritis Care Res 55:985–989. https://doi.org/10.1002/art.22359

    Article  Google Scholar 

  23. McCormick HM, Neubuerger KT (1958) Giant-cell arteritis involving small meningeal and intracerebral vessels. J Neuropathol Exp Neurol 17:471–478

    Article  CAS  Google Scholar 

  24. Gilden D, White T, Khmeleva N et al (2015) Prevalence and distribution of VZV in temporal arteries of patients with giant cell arteritis. Neurology:1–8

    Google Scholar 

  25. Pisapia DJ, Lavi E (2016) VZV, temporal arteritis, and clinical practice: false positive immunohistochemical detection due to antibody cross-reactivity. Exp Mol Pathol 100:114–115. https://doi.org/10.1016/j.yexmp.2015.12.007

    Article  CAS  PubMed  Google Scholar 

  26. Everett CM, Graves TD, Lad S et al (2008) Aggressive CNS lupus vasculitis in the absence of systemic disease activity [5]. Rheumatology 47:107–109. https://doi.org/10.1093/rheumatology/kem264

    Article  CAS  PubMed  Google Scholar 

  27. Ramos-Casals M, Nardi N, Lagrutta M et al (2006) Vasculitis in systemic lupus erythematosus. Lupus 85:95–104. https://doi.org/10.1097/01.md.0000216817.35937.70

    Article  Google Scholar 

  28. Ramos-Casals M, Solans R, Rosas J et al (2008) Primary Sjögren syndrome in Spain. Medicine (Baltimore) 87:210–219. https://doi.org/10.1097/MD.0b013e318181e6af

    Article  CAS  Google Scholar 

  29. Caballol Pons N, Montalà N, Valverde J et al (2010) Isolated cerebral vasculitis associated with rheumatoid arthritis. Jt Bone Spine 77:361–363. https://doi.org/10.1016/j.jbspin.2010.02.030

    Article  Google Scholar 

  30. Niemelä RK, Hakala M (1999) Primary Sjögren’s syndrome with severe central nervous system disease. Semin Arthritis Rheum 29:4–13

    Article  Google Scholar 

  31. Ma WL, Li CC, Yu SC, Tien HF (2014) Adult T-cell lymphoma/leukemia presenting as isolated central nervous system T-cell lymphoma. Case Rep Hematol 2014:917369. https://doi.org/10.1155/2014/917369

    Article  PubMed  PubMed Central  Google Scholar 

  32. Ponzoni M, Ferreri AJM, Campo E et al (2007) Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol 25:3168–3173. https://doi.org/10.1200/JCO.2006.08.2313

    Article  PubMed  Google Scholar 

  33. Song DK, Boulis NM, McKeever PE, Quint DJ (2002) Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis. Am J Neuroradiol 23:239–242

    PubMed  PubMed Central  Google Scholar 

  34. Schaafsma JD, Hui F, Wisco D et al (2016) High-resolution vessel wall MRI: appearance of intravascular lymphoma mimics central nervous system vasculitis. Clin Neuroradiol:1–4. https://doi.org/10.1007/s00062-016-0529-9

  35. Berlit P (2009) Isolated angiitis of the CNS and bacterial endocarditis: similarities and differences. J Neurol 256:792–795. https://doi.org/10.1007/s00415-009-5018-5

    Article  PubMed  Google Scholar 

  36. Calabrese LH, Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 67:20–39

    Article  CAS  Google Scholar 

  37. Birnbaum J, Hellmann DB (2009) Primary angiitis of the central nervous system. Arch Neurol 66:704–709

    Article  Google Scholar 

  38. Alhalabi M, Moore PM (1994) Serial angiography in isolated angiitis of the central nervous system. Neurology 44:1221–1226. https://doi.org/10.1212/WNL.44.7.1221

    Article  CAS  PubMed  Google Scholar 

  39. Ferris EJ, Levine HL (1973) Cerebral arteritis: classification. Radiology 109:327–341. https://doi.org/10.1148/109.2.327

    Article  CAS  PubMed  Google Scholar 

  40. Vollmer TL, Guarnaccia J, Harrington W et al (1993) Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges. Arch Neurol 50:925–930. https://doi.org/10.1001/archneur.1993.00540090032007

    Article  CAS  PubMed  Google Scholar 

  41. Kadkhodayan Y, Alreshaid A, Moran CJ et al (2004) Primary angiitis of the central nervous system at conventional angiography. Radiology 233:878–882. https://doi.org/10.1148/radiol.2333031621

    Article  PubMed  Google Scholar 

  42. Mossa-Basha M, Hwang WD, De Havenon A et al (2015) Multicontrast high-resolution vessel wall magnetic resonance imaging and its value in differentiating intracranial vasculopathic processes. Stroke 46:1567–1573. https://doi.org/10.1161/STROKEAHA.115.009037

    Article  PubMed  Google Scholar 

  43. Obusez EC, Hui F, Hajj-Ali RA et al (2014) High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis. AJNR Am J Neuroradiol 35:1527–1532. https://doi.org/10.3174/ajnr.A3909

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Ahn S-H, Lee J, Kim Y-J et al (2015) Isolated MCA disease in patients without significant atherosclerotic risk factors: a high-resolution magnetic resonance imaging study. Stroke 46:697–703. https://doi.org/10.1161/STROKEAHA.114.008181

    Article  CAS  PubMed  Google Scholar 

  45. Ryoo S, Cha J, Kim SJ et al (2014) High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke 45:2457–2460. https://doi.org/10.1161/STROKEAHA.114.004761

    Article  PubMed  Google Scholar 

  46. Aviv RI, Benseler SM, Silverman ED et al (2006) MR imaging and angiography of primary CNS vasculitis of childhood. Am J Neuroradiol 27:192–199. 27/1/192 [pii]

    CAS  PubMed  PubMed Central  Google Scholar 

  47. Pomper MG, Miller TJ, Stone JH et al (1999) CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography. Am J Neuroradiol 20:75–85

    CAS  PubMed  Google Scholar 

  48. Powers WJ (2015) Primary angiitis of the central nervous system. Diagnostic criteria. Neurol Clin 33:515–526. https://doi.org/10.1016/j.ncl.2014.12.004

    Article  PubMed  Google Scholar 

  49. Salvarani C, Brown RD, Calamia KT et al (2011) Primary central nervous system vasculitis presenting with intracranial hemorrhage. Arthritis Rheum 63:3598–3606. https://doi.org/10.1002/art.30594

    Article  PubMed  Google Scholar 

  50. Salvarani C, Brown RD, Calamia KT et al (2008) Primary central nervous system vasculitis with prominent leptomeningeal enhancement: a subset with a benign outcome. Arthritis Rheum 58:595–603. https://doi.org/10.1002/art.23300

    Article  PubMed  Google Scholar 

  51. Miller DV, Salvarani C, Hunder GG et al (2009) Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 33:35–43. https://doi.org/10.1097/PAS.0b013e318181e097

    Article  PubMed  Google Scholar 

  52. Torres J, Loomis C, Cucchiara B et al (2016) Diagnostic yield and safety of brain biopsy for suspected primary central nervous system angiitis. Stroke 47:2127–2129. https://doi.org/10.1161/STROKEAHA.116.013874

    Article  PubMed  Google Scholar 

  53. Alrawi A, Trobe JD, Blaivas M, Musch DC (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53:852–855. https://doi.org/10.1212/WNL.53.4.852

    Article  Google Scholar 

  54. Salvarani C, Brown RD, Calamia KT et al (2008) Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology 47:1671–1677. https://doi.org/10.1093/rheumatology/ken328

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Weill Cornell Medical College, New York, NY, USA

    Aaron M. Gusdon, Mackenzie P. Lerario & Gary L. Bernardini

  2. NewYork-Presbyterian Queens, Flushing, NY, USA

    Mackenzie P. Lerario & Gary L. Bernardini

  3. Fordham University Graduate School of Social Service, New York, NY, USA

    Mackenzie P. Lerario

  4. Department of Pathology, Weill Cornell Medical College, New York, NY, USA

    Ehud Lavi

Authors
  1. Aaron M. Gusdon
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mackenzie P. Lerario
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ehud Lavi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gary L. Bernardini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mackenzie P. Lerario .

Editor information

Editors and Affiliations

  1. Associate Chair of Neurology for Research, New York Medical College, Associate Professor of Neurology, Neurosurgery and Radiology, New York Medical College, Director of the Neuroendovascular Surgery Fellowship, Neuroendovascular Surgery (Interventional Neurologist) Attending Medical Director of Neurocritical Care, Westchester Medical Center at New York Medical College, New York, USA

    Fawaz Al-Mufti, MD

  2. Division of Neurointerventional Radiology, Goodman Campbell Brain and Spine, Division of Neurointerventional Radiology, Ascension St. Vincent Medical Center, Department of Neurosurgery, Marian College of Osteopathic Medicine, Indianapolis, IN, USA

    Krishna Amuluru MD

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Science+Business Media, LLC, part of Springer Nature

About this protocol

Check for updates. Verify currency and authenticity via CrossMark

Cite this protocol

Gusdon, A.M., Lerario, M.P., Lavi, E., Bernardini, G.L. (2021). Central Nervous System Vasculitis: Primary Angiitis of the Central Nervous System. In: Al-Mufti, MD, F., Amuluru MD, K. (eds) Cerebrovascular Disorders. Neuromethods, vol 170. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-1530-0_21

Download citation

  • DOI: https://doi.org/10.1007/978-1-0716-1530-0_21

  • Published:

  • Publisher Name: Humana, New York, NY

  • Print ISBN: 978-1-0716-1529-4

  • Online ISBN: 978-1-0716-1530-0

  • eBook Packages: Springer Protocols

Publish with us

Policies and ethics

Search

Navigation