Zusammenfassung
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.
Abstract
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.
Literatur
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
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
Alrawi A, Trobe JD, Blaivas M et al. (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53:858–860
Berlit P (1997) Giant cell arteritis. In: Lechtenberg R, Schutta HS (eds) Practice guidelines for neurologic therapy. Dekker, New York
Berlit P (1994) The spectrum of vasculopathies in the differential diagnosis of vasculitis. Semin Neurol 14:370–379
Berlit P, Steinbrecher A (2002) Zerebrale Vaskulitis. Fortschr Neurol Psychiatr 70:663–677
Berlit P (2004) Zur Diagnose und Differenzialdiagnose der zerebralen Vaskulitis. Nervenarzt 75:105–112
Chu CT, Gray L, Goldstein LB et al. (1998) Diagnosis of intracranial vasculitis: a multi-disciplinary approach. J Neuropathol Exp Neurol 57:30–38
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
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
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
International Study Group for Behçet’s disease (1990) Criteria for Behçet’s disease. Lancet 335:1078–1080
Jennette JC, Falk RJ, Andrassy K et al. (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192
Moore P, Richardson B (1998) Neurology of the vasculitides and connective tissue diseases. J Neurol Neurosurg Psychiat 65:10–22
Moore PM (1989) Diagnosis and management of isolated angiitis of the central nervous system. Neurology 39:167–173
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
Ruiz-Irastorza G, Khamashta MA, Castellino G et al. (2001) Systemic lupus erythematosus. Lancet 357:1027–1032
Schmidley JW (2000) Central nervous system angiitis. Butterworth, Boston
Specks U, Moder KG, McDonald TJ (2000) Meningeal involvement in Wegener granulomatosis. Mayo Clin Proc 75:856–859
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
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
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Berlit, P. Zerebrale Vaskulitis . Nervenarzt 75, 817–830 (2004). https://doi.org/10.1007/s00115-004-1750-3
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DOI: https://doi.org/10.1007/s00115-004-1750-3