Medscape is available in 5 Language Editions – Choose your Edition here.


Primary Angiitis of the CNS Follow-up

  • Author: Mazen Noufal, MD; Chief Editor: Helmi L Lutsep, MD  more...
Updated: Oct 24, 2014

Further Outpatient Care

See the list below:

  • When initially abnormal, brain MRI and CSF examination can be used with clinical findings to evaluate patient’s response to treatment.
  • Patients taking cyclophosphamide should be evaluated periodically for signs of drug toxicity. CBC should be performed every 2 weeks. The dose should be reduced or the drug should be temporarily held in case of leucopenia (WBC < 4000) or absolute neutropenia (neutrophils < 2000).
  • Patients, especially elderly patients, taking steroids should be given vitamin D and calcium supplements for bone mineral loss, and H2 blockers or proton-pump inhibitors for peptic ulcer disease. Blood glucose should also be monitored closely to detect iatrogenic diabetes.

Further Inpatient Care

See the list below:

  • Other comorbidities such as hypertension, diabetes, hypercholesterolemia, and heart diseases should be treated appropriately.
  • Antiplatelet aggregation agents should be given to patients with ischemic lesions, unless contraindicated.
  • Confusion, agitation, seizures, headache, nausea, and vomiting should be treated symptomatically.


Complications for primary angiitis of the CNS are nonspecific. Patients who are immobile due to neurologic deficits are at higher risk for pneumonia, decubitus ulcers, and deep venous thrombosis (DVT). Complications of steroids and immunosuppressants are mentioned above in Further Outpatient Care.



Prognosis of primary CNS angiitis is usually guarded, even with appropriate therapy. Prognosis is good in benign angiopathy of the CNS and postpartum CNS angiopathy, but these diagnoses are usually confirmed only in retrospect.


Patient Education

See the list below:

  • Patients should be educated about the chronic nature of the disease, and about the potential toxic side effects of steroids and immunosuppressants.
  • Patients should also be educated about the importance of controlling other cerebrovascular risk factors, such as hypertension, diabetes, hyperlipidemia, and smoking.
Contributor Information and Disclosures

Mazen Noufal, MD Neurologist, Baptist Health Medical Center

Mazen Noufal, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.


James W Schmidley, MD Professor of Neurology, Virginia Tech Carilion School of Medicine

James W Schmidley, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Neurological Association, Phi Beta Kappa

Disclosure: Received stipend from Oakstone medical publishing for review panel membership.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association

Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2.

Additional Contributors

Christopher Luzzio, MD Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

  1. Crowe WE. Immunopathogenesis and Diagnosis. Immunopathogenesis of Vasculitis. Littleton, MA: PSG Publishing Company; 1988. Chapter 1, Section 1.

  2. Schmidley, James W. Central Nervous System Angiitis. USA: Butterworth-Heinemann; 2000. Chapters 1, 2, 3, 4.

  3. Younger DS. Vasculitis of the nervous system. Curr Opin Neurol. 2004 Jun. 17(3):317-36. [Medline].

  4. Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum. 1997 Jul. 40(7):1189-201. [Medline].

  5. Moskowitz MA, Henrikson BM. Trigeminovascular neurons and the arteritis complicating herpes zoster ophthalmicus. Neurology. 1985 Mar. 35(3):444. [Medline].

  6. F G. Joseph, N J. Scolding. Cerebral Vasculitis: A Practical Approach. Practical Neurology. 2002. 2:80.

  7. Miller DV, Salvarani C, Hunder GG, Brown RD, Parisi JE, Christianson TJ. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol. 2009 Jan. 33(1):35-43. [Medline].

  8. Benseler SM. Central nervous system vasculitis in children. Curr Rheumatol Rep. 2006 Dec. 8(6):442-9. [Medline].

  9. Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis with prominent leptomeningeal enhancement: a subset with a benign outcome. Arthritis Rheum. 2008 Feb. 58(2):595-603. [Medline].

  10. Katsetos CD, Poletto E, Kasmire KE, Walleigh D, Kumar I, Pascasio JM, et al. Childhood primary angiitis of the central nervous system with metachronous hemorrhagic infarcts: a postmortem study with clinicopathologic correlation. Semin Pediatr Neurol. 2014 Jun. 21(2):184-94. [Medline].

  11. Wiszniewska M, Devuyst G, Bogousslavsky J. Giant cell arteritis as a cause of first-ever stroke. Cerebrovasc Dis. 2007. 24(2-3):226-30. [Medline].

  12. Salvarani C, Brown RD Jr, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement. Neurology. 2008 Jun 10. 70(24 Pt 2):2394-400. [Medline].

  13. Elbers J, Halliday W, Hawkins C, Hutchinson C, Benseler SM. Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol. 2010 Nov. 68(5):602-10. [Medline].

  14. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007 Nov. 62(5):442-51. [Medline].

  15. Scolding NJ, Jayne DR, Zajicek JP, et al. Cerebral vasculitis--recognition, diagnosis and management. QJM. 1997 Jan. 90(1):61-73. [Medline].

  16. Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke. 1988 Sep. 19(9):1159-70. [Medline].

  17. Hajj-Ali RA, Furlan A, Abou-Chebel A, et al. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum. 2002 Dec 15. 47(6):662-9. [Medline].

  18. White ML, Hadley WL, Zhang Y, et al. Analysis of central nervous system vasculitis with diffusion-weighted imaging and apparent diffusion coefficient mapping of the normal-appearing brain. AJNR Am J Neuroradiol. 2007 May. 28(5):933-7. [Medline].

  19. Singh S, John S, Joseph TP, et al. Primary angiitis of the central nervous system: MRI features and clinical presentation. Australas Radiol. 2003 Jun. 47(2):127-34. [Medline].

  20. Rosati A, Pianigiani N, Pagnini I, Guerrini R, Cimaz R, Simonini G. Sequential MR angiography in childhood primary angiitis of the CNS. Pediatr Neurol. 2013 Aug. 49(2):127-9. [Medline].

  21. Kadkhodayan Y, Alreshaid A, Moran CJ, et al. Primary angiitis of the central nervous system at conventional angiography. Radiology. 2004 Dec. 233(3):878-82. [Medline].

  22. Alrawi A, Trobe JD, Blaivas M, et al. Brain biopsy in primary angiitis of the central nervous system. Neurology. 1999 Sep 11. 53(4):858-60. [Medline].

  23. Kolodny EH, Rebeiz JJ, Caviness VS Jr, et al. Granulomatous angiitis of the central nervous system. Arch Neurol. 1968 Nov. 19(5):510-24. [Medline].

  24. Schwab P, Lidov HG, Schwartz RB, Anderson RJ. Cerebral amyloid angiopathy associated with primary angiitis of the central nervous system: report of 2 cases and review of the literature. Arthritis Rheum. 2003 Jun 15. 49(3):421-7. [Medline].

  25. Scolding NJ, Joseph F, Kirby PA, Mazanti I, Gray F, Mikol J, et al. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain. 2005 Mar. 128:500-15. [Medline].

  26. Calabrese LH, Gragg LA, Furlan AJ. Benign angiopathy: a distinct subset of angiographically defined primary angiitis of the central nervous system. J Rheumatol. 1993 Dec. 20(12):2046-50. [Medline].

  27. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol. 2009 Oct. 9(5):256-67. [Medline].

  28. Duna GF, Calabrese LH. Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system. J Rheumatol. 1995 Apr. 22(4):662-7. [Medline].

  29. Lie JT. Classification and histopathologic spectrum of central nervous system vasculitis. Neurol Clin. 1997 Nov. 15(4):805-19. [Medline].

Lateral right internal carotid angiogram shows beading of anterior cerebral arteries (arrowheads) and beading (straight arrows), segmental dilatation (curved arrow), and narrowing of middle cerebral arteries (open arrow).
Low power view of inflamed vessel in the subarachnoid space shows fibrinoid necrosis (pink areas) superiorly.
Higher power view of small inflamed vessel in the medulla, with fibrinoid necrosis in the wall and chronic transmural inflammation.
(a) T1-weighted left parasagittal MRI showing hypointense lesions in the cortical and subcortical white matter of the left superior temporal gyrus and frontal and frontoparietal operculum with sulcal obliteration. (b) The postgadolinium MRI at this level showing nodular parenchymal and linear pial enhancement.
(a) T2-weighted axial MRI showing hyperintense lesions in the lentiform nuclei, head of the caudate nuclei and frontoparietal subcortical white matter. (b) On T1-weighted (postgadolinium) axial image, the lentiform nuclei and head of caudate nuclei are heterogeneously hypointense, while subcortical white matter lesions are isointense. Linear areas of pial enhancement are seen bilaterally.
(a) T2-weighted axial MRI showing large, well-defined hyperintense lesions in the left frontal and parietal lobes. (b) Postgadolinium T1-weighted axial MRI showing nodular parenchymal and cortical-pial enhancement of the lesion. A follow up (after 3 mo) T2-weighted axial (c) and postgadolinium (d) T1-weighted axial MRIs showing significant reduction of the lesion size and nodular enhancing areas.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.