Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndromes

  • Leonard H. Calabrese
  • Aneesh B. Singhal


Vasculitis that exclusively involves the central nervous system (CNS) is among the most complex and poorly understood forms of vascular inflammatory disease. The variable terminology for CNS vasculitis continues to pose significant challenges. CNS vasculitis that is not associated with a known underlying cause or some form of systemic vasculitis is termed primary angiitis of the CNS (PACNS). Some cases of PACNS confirmed by histopathology have granulomatous features of inflammation. The term “granulomatous inflammation of the nervous system” has been used for such cases. For practical purposes, now GANS and PACNS are considered to be largely synonymous terms. PACNS presents in a subacute manner, with an elapse of many months between the time of first symptom onset and diagnostic confirmation. The clinical hallmarks of PACNS are headache, slowly-evolving encephalopathy, and multi-focal strokes. Fever, other constitutional symptoms, and extra-CNS manifestations are absent. Acute phase reactants tend to be normal in PACNS. Lumbar puncture typically reveals a lymphocytic pleocytosis.


Posterior Reversible Encephalopathy Syndrome Brain Biopsy Reversible Posterior Leukoencephalopathy Syndrome Pituitary Apoplexy Reversible Cerebral Vasoconstriction Syndrome 
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  1. Alrawi A, Trobe JD, Blaivas M, Musch DC. Brain biopsy in primary angiitis of the central nervous system. Neurology 1999;53:858–60PubMedCrossRefGoogle Scholar
  2. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146:34–44PubMedCrossRefGoogle Scholar
  3. Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum. 1997;40:1189–201PubMedGoogle Scholar
  4. Chen SP, Fuh JL, Chang FC, Lirng JF, Shia BC, Wang SJ. Transcranial color Doppler study for reversible cerebral vasoconstriction syndromes. Ann Neurol. 2008;63:751–7PubMedCrossRefGoogle Scholar
  5. Chen SP, Fuh JL, Lirng JF, Chang FC, Wang SJ. Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology 2006;67:2164–9PubMedCrossRefGoogle Scholar
  6. Chu CT, Gray L, Goldstein LB, Hulette CM. Diagnosis of intracranial vasculitis: a multi-disciplinary approach. J Neuropathol Exp Neurol. 1998;57:30–8PubMedCrossRefGoogle Scholar
  7. Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101PubMedCrossRefGoogle Scholar
  8. Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum. 2002;47: 662–9PubMedCrossRefGoogle Scholar
  9. Molloy ES, Singhal AB, Calabrese LH. Tumor-like mass lesion – an under-recognized presentation of primary angiitis of the central nervous system. Ann Rheum Dis. 2008;67:1732–5PubMedCrossRefGoogle Scholar
  10. Moore PM. Central nervous system vasculitis. Curr Opin Neurol. 1998;11:241–6PubMedCrossRefGoogle Scholar
  11. Parisi JE, Moore PM. The role of biopsy in vasculitis of the central nervous system. Semin Neurol. 1994;14:341–8PubMedCrossRefGoogle Scholar
  12. Schwedt TJ, Matharu MS, Dodick DW. Thunderclap headache. Lancet Neurol. 2006;5:621–31PubMedCrossRefGoogle Scholar
  13. Singhal AB. Cerebral vasoconstriction without subarachnoid blood: associated conditions, clinical and neuroimaging characteristics. Ann Neurol. 2002:S:59–60Google Scholar
  14. Singhal AB. Postpartum angiopathy with reversible posterior leukoen-cephalopathy. Arch Neurol. 2004;61:411–16PubMedCrossRefGoogle Scholar
  15. Singhal AB, Caviness VS, Begleiter AF, Mark EJ, Rordorf G, Koroshetz WJ. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology 2002;58:130–3PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • Leonard H. Calabrese
    • 1
  • Aneesh B. Singhal
    • 2
  1. 1.Cleveland Clinic FoundationDepartment of Rheumatology & ImmunologyClevelandUSA
  2. 2.Neurology, MGH Stroke Research CenterHarvard Medical School, Massachusetts General HospitalBostonUSA

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