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

Epidemiology: 1) average age of onset 45 years 2) slight male predominance 3) rare disorder [5] Pathology: 1) CNS vasculitis, a granulomatous inflammatory process [2] a) centered around small veins & arterioles b) more prominent in leptomeninges than underlying cortex c) 15% of patients without granulomatous process 2) inflammation is patchy in distribution 3) brain & spinal cord may be involved Clinical manifestations: 1) chronic & fluctuating course 2) headache associated with nausea & vomiting (most common presentation) - recurrent headaches [2] 3) neurologic manifestations a) non-focal neurologic symptoms - progressive encephalopathy - cognitive deterioration [3] - deterioration may occur over a period of days [2] - confusion, drowsiness coma - may interrupt prolonged periods of clinical remission b) acute stroke-like presentations may occur - transient ischemic attack - ischemic stroke c) cerebral hemorrhage < 4% d) focal neurologic signs e) non-focal neurologic signs often coexist (50%) f) seizures (25%) 4) systemic manifestations (< 20%) - fever, weight loss, arthralgias, myalgias Laboratory: 1) cerebrospinal fluid is abnormal (90%) 2) erythrocyte sedimentation rate (ESR) is generally normal Special laboratory: - lumbar puncture - biopsy of CNS tissue, including leptomeninges - granulomatous vasculitis Radiology: 1) cerebral angiography a) 40% of patients with biopsy-proven CNS vasculitis have negative cerebral angiography b) 25% of patients with angiographically demonstrable CNS vasculitis have negative biopsy 2) computed tomography is neither sensitive not specific 3) magnetic resonance imaging does not distinguish from other vasculopathic or demyelinating conditions Differential diagnosis: 1) Cogan's syndrome 2) Behcet's syndrome 3) systemic lupus erythematosus 4) polyarteritis 5) cocaine-induced vasculopathy 6) demyelinating disease: multiple sclerosis 7) HIV infection 8) Lyme disease 9) syphilis 10) carcinomatous meningitis 11) angiocentric immunoproliferative lesions 12) antiphospholipid syndrome 13) reversible cerebral vasoconstriction (81% women) Management: 1) glucocorticoids 2) nimodipine to prevent vasospasm 3) cyclophosphamide & high-dose glucocorticoids [2] 4) 6 months of intravenous cyclophosphamide & glucocorticoid therapy, followed by 18 months of mycophenolate mofetil or azathioprine [2,3] 5) high mortality among patients with severe disease not treated with cytotoxic agents

General

vasculitis arteritis encephalopathy neuroinflammatory disease

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 846
  2. 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. 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; PMID: 20889380 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. 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: 24782189
  6. Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009 Jun;66(6):704-9. PMID: 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: 25399939