Vasculitis is an inflammation of blood vessels due to various origins. Vessels of the peripheral and/or central nervous systems may be involved. Clinical and radiological diagnosis of cerebral vasculitis is challenging as there is no vasculitis-specific symptom or imaging sign. Furthermore, it may mimic other neurological diseases, which would require different treatment approaches. Neuroimaging of cerebral vasculitis should begin with initial MRI to assess the degree of parenchymal damage and to detect vessel wall changes. If the results are indefinite DSA should be pursued in order to detect potential changes of medium-sized vessels. Vasculitides of small vessels cannot be detected by vascular imaging and require brain or leptomeningeal biopsy.
KeywordsSystemic Lupus Erythematosus Hemolytic Uremic Syndrome Giant Cell Arteritis Central Nervous System Involvement Takayasu Arteritis
- Giannini C, Salvarani C, Hunder G, Brown R (2012) Primary central nervous system vasculitis: pathology and mechanisms. Acta Neuropathol 123(6):759–772 (*this recent review by authors from the Mayo Clinic gives a very detailed overview about clinical and imaging signs of PCNSV)Google Scholar
- Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG et al (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum, vol 37, pp 187–192Google Scholar
- Pipitone N, Versari A, Salvarani C (2008) Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford) 47:403–408Google Scholar
- Salvarani C, Brown RD Jr, Hunder GG (2012) Adult primary central nervous system vasculitis: an update. Curr Opin Rheumatol 24:46–52 (*A nice review providing an extensive description of several subsets of PCNSV including neuroimaging and disease management) Google Scholar
- Sattar A, Manousakis G, Jensen MB (2010) Systematic review of reversible cerebral vasoconstriction syndrome. Expert Rev Cardiovasc Ther 8(10):1417–1421Google Scholar
- Wengenroth M, Hoeltje J, Repenthin J, Meyer TN, Bonk F, Becker H, Faiss S, Stammel O, Urban PP, Bruening R (2013) Central nervous system involvement in adults with epidemic hemolytic uremic syndrome (HUS). AJNR Google Scholar