Skip to main content

Zerebrale Vaskulitis

Cerebral vasculitis


Zerebrale Vaskulitiden sind im klinischen Alltag des Neurologen selten, es ist jedoch wichtig, in der Differenzialdiagnose (z. B. bei Arteriitis cranialis, Kollagenosen, Behçet-Syndrom, Wegener-Granulomatose, Churg-Strauss-Syndrom, Lupus erythematodes, Sjögren-Syndrom) an sie zu denken. Leitsymptome sind, in variabler Kombination, Kopfschmerzen, multifokale Ausfälle und Enzephalopathie. Charakteristisch für systemische Vaskulitiden sind akute Entzündungszeichen und die Kombination mit peripher neurologischen Manifestationen (oft schmerzhafte Mononeuropathia multiplex) sowie Manifestationen an anderen Organen (Gelenke, Haut, Augen, Nieren, Lunge, Herz). Bei der isolierten ZNS-Vaskulitis ist ein pathologischer Liquorbefund entscheidend. Wegweisend in der Diagnostik sind bildgebende Verfahren, Liquor- und Serumbefunde; stets ist die histologische Absicherung der Diagnose erforderlich.


Cerebral vasculitides are infrequent in neurological practice but important for the differential diagnosis of multifocal CNS symptoms. The clinical presentation is typically a combination of chronic headache, multiple strokes and encephalopathy. In systemic vasculitis, a combination of CNS symptoms with inflammatory signs and with peripheral nervous system symptoms (painful multiplex polyneuropathy) or other organ manifestations (skin, joints, kidney, heart, lungs, eye) are frequent. In isolated CNS angiitis, CSF examinations are of particular value. Besides MRI, angiography, CSF and serum examinations, the verification of the diagnosis by biopsy is mandatory.

This is a preview of subscription content, access via your institution.

Abb. 1
Abb. 2
Abb. 3


  1. ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature (1999) The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus. Arthritis Rheum 42:599–608

    Article  PubMed  Google Scholar 

  2. Akman-Demir G, Serdaroglu P, Tasci B (1999) Clinical patterns of neurological involvement in Behcet’s disease: evaluation of 200 patients. The Neuro-Behcet Study Group. Brain 122:2171–2182

    Article  PubMed  Google Scholar 

  3. Alrawi A, Trobe JD, Blaivas M et al. (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53:858–860

    CAS  PubMed  Google Scholar 

  4. Berlit P (1997) Giant cell arteritis. In: Lechtenberg R, Schutta HS (eds) Practice guidelines for neurologic therapy. Dekker, New York

  5. Berlit P (1994) The spectrum of vasculopathies in the differential diagnosis of vasculitis. Semin Neurol 14:370–379

    CAS  Google Scholar 

  6. Berlit P, Steinbrecher A (2002) Zerebrale Vaskulitis. Fortschr Neurol Psychiatr 70:663–677

    Article  CAS  Google Scholar 

  7. Berlit P (2004) Zur Diagnose und Differenzialdiagnose der zerebralen Vaskulitis. Nervenarzt 75:105–112

    Article  CAS  PubMed  Google Scholar 

  8. Chu CT, Gray L, Goldstein LB et al. (1998) Diagnosis of intracranial vasculitis: a multi-disciplinary approach. J Neuropathol Exp Neurol 57:30–38

    CAS  PubMed  Google Scholar 

  9. Duna GF, Calabrese LH (1995) Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system. J Rheumatol 22:662–667

    CAS  PubMed  Google Scholar 

  10. Fauci AS, Haynes BF, Katz P et al. (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98:76–85

    CAS  PubMed  Google Scholar 

  11. Gayraud M, Guillevin L, le Toumelin P et al. (2001) Long-term follow up of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 44:666–675

    Article  CAS  PubMed  Google Scholar 

  12. International Study Group for Behçet’s disease (1990) Criteria for Behçet’s disease. Lancet 335:1078–1080

    PubMed  Google Scholar 

  13. Jennette JC, Falk RJ, Andrassy K et al. (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192

    CAS  PubMed  Google Scholar 

  14. Moore P, Richardson B (1998) Neurology of the vasculitides and connective tissue diseases. J Neurol Neurosurg Psychiat 65:10–22

    CAS  PubMed  Google Scholar 

  15. Moore PM (1989) Diagnosis and management of isolated angiitis of the central nervous system. Neurology 39:167–173

    CAS  PubMed  Google Scholar 

  16. Nishino H, Rubino FA, DeRemee RA et al. (1993) Neurological involvement in Wegener’s granulomatosis. An analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 33:4–9

    CAS  PubMed  Google Scholar 

  17. Ruiz-Irastorza G, Khamashta MA, Castellino G et al. (2001) Systemic lupus erythematosus. Lancet 357:1027–1032

    Article  CAS  PubMed  Google Scholar 

  18. Schmidley JW (2000) Central nervous system angiitis. Butterworth, Boston

  19. Specks U, Moder KG, McDonald TJ (2000) Meningeal involvement in Wegener granulomatosis. Mayo Clin Proc 75:856–859

    CAS  PubMed  Google Scholar 

  20. Stone JH, Pomper MG, Roubenoff R et al. (1994) Sensitivities of noninvasive tests for central nervous system vasculitis: a comparison of lumbar puncture, computed tomography, and magnetic resonance imaging. J Rheumatol 21:1277–1282

    CAS  PubMed  Google Scholar 

  21. Wechsler B, Dell’Isola B, Vidailhet M et al. (1993) MRI in 31 patients with Behçet’s disease and neurological involvement. Prospective study with clinical correlation. J Neurol Neurosurg Psychiatry 56:793–798

    CAS  PubMed  Google Scholar 

Download references


Keine Angaben

Author information

Authors and Affiliations


Corresponding author

Correspondence to P. Berlit.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Berlit, P. Zerebrale Vaskulitis . Nervenarzt 75, 817–830 (2004).

Download citation

  • Issue Date:

  • DOI:


  • Behçet-Syndrom
  • Wegener-Granulomatose
  • Churg-Strauss-Syndrom
  • Lupus erythematodes
  • Sjögren-Syndrom


  • Behçet syndrome
  • Wegener granulomatosis
  • Churg-Strauss syndrome
  • Lupus erythematosus
  • Sjögren syndrome