isolated (primary) central nervous system vasculitis (angiitis) (PACNS)

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Epidemiology

  • average age of onset 45 years
  • slight male predominance
  • rare disorder[5]

Pathology

Clinical manifestations

Laboratory

Diagnostic procedures

Radiology

Differential diagnosis

Management

More general terms

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 846
  2. 2.0 2.1 2.2 2.3 2.4 2.5 Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2015, 2018, 2022.
    Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. 3.0 3.1 3.2 Weiner S, In: UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
  4. Hutchinson C et al Treatment of small vessel primary CNS vasculitis in children: An open-label cohort study. Lancet Neurol 2010 Oct 4; <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/20889380 <Internet> http://neurology.jwatch.org/cgi/content/full/2010/1012/2?q=pfw-featured http://dx.doi.org/10.1016/S1474-4422(10)70243-X
  5. 5.0 5.1 de Boysson H et al. 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 Rheum 2014 May; 66:1315 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24782189
  6. Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009 Jun;66(6):704-9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19506130
  7. Rodriguez-Pla A, Monach PA. Primary angiitis of the central nervous system in adults and children. Rheum Dis Clin North Am. 2015;41(1):47-62, viii. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25399939