Central nervous system vasculitis

Vasculitides are a very heterogeneous group of diseases with a wide range of clinical presentations. Due to inflammatory changes in the vessel wall, distal tissue is jeopardized by ischaemia (vascular occlusion, embolization) or, less frequently, vessel rupture and bleeding can occur at the site of the injury. Clinically, three basic presentations of CNS vasculitis manifestations can be specified: acute territorial ischaemic stroke, i.e. involvement of large arteries with a typical presentation consistent with the territory affected, but often with accompanying atypical symptoms in the medical history (cephalalgia, encephalopathy, or epileptic seizures); "atypical multiple sclerosis (MS-plus)" with involvement of small arteries, having a relapsing-remitting course with atypical MS symptoms (epileptic seizures, headaches); and acute encephalopathy with progressive disturbance of consciousness. In CNS vasculitides, immunosuppressive therapy always has to be supplemented by antiplatelet or anticoagulant therapy. Vascular risks are to be managed aggressively (high-dose statins, antihypertensive drugs, diabetes control). The diagnosis of CNS vasculitis should be taken into consideration despite its very low incidence, given the options of targeted therapy with a good chance of improving the prognosis in affected patients.

Keywords: vasculitis, ischaemic stroke, primary CNS vasculitis