Isolated central nervous system vasculitis (ICNSV) is a vasculitis limited to the central nervous system. Its etiology and pathogenesis has not been exactly elucidated yet. It is predominantly seen in the fourth to sixth decades. Neurological symptoms in ICNSV can manifest in a broad spectrum, ranging from headache to stroke. A high degree of clinical suspicion is of great importance for diagnosis. Although there is no definitive diagnostic laboratory / serological test for ICNSV; laboratory tests are performed in patients with ICNSV to exclude secondary causes. CSF analysis usually demonstrates a high protein content and lymphocytic pleocytosis. Imaging findings are quite variable, ranging from small ischemic changes to large areas of infarction, hemorrhage, white matter edema also contrast enhancement. Magnetic resonance imaging is the most commonly used imaging modality in the workup of patients with suspected ICNSV. Vessel wall imaging MR technique, which has been used frequently in recent years, can be helpful in the diagnosis of ICNSV, determination of the appropriate biopsy location and follow-up after treatment. Cerebral digital subtraction angiography is considered the most sensitive imaging modality for the diagnosis of ICNSV; but the findings are not pathognomonic. Histological confirmation obtained with cerebral and meningeal biopsy samples is the gold standard for the definitive diagnosis of ICNSV. In this chapter, we discuss the ICNSV in detail with a diagnostic algorithm in the light of modalities that can be used in diagnosis

Cervical artery dissection (CAD) is characterized by an intramural haematoma due to a subintimal tear and accounts for up to 25% of ischaemic strokes in young and middle-aged adults. Data regarding intravenous thrombolysis and endovascular thrombectomy in CAD are scarce and observational – both are reasonably safe and probably recommended. Based on observational evidence, antithrombotic therapy is used to prevent first or recurrent cerebral ischaaemic events in acute or subacute CAD, and event rates are low with either antiplatelet or anticoagulant therapy. The long-term rate of recurrent cerebral ischaemic events or bleeding complications in CAD patients is small while under antithrombotic treatment. Cerebral vasculitis treatment is based on observational series. When primary angiitis of the central nervous system is confirmed by biopsy, a combination of glucocorticoids and cyclophosphamide should be started. Rituximab may be used in patients who are intolerant of cyclophosphamide. In atypical, non-biopsy-proven cases, treatment should be adapted to the severity of neurological involvement. For giant cell arteritis, initial high-dose prednisolone is recommended, beginning a slow taper after 2–4 weeks and continuing at a low dose for 1–2 years. Treatment of p-ANCA-positive and -negative systemic vasculitis with cerebral involvement includes induction corticosteroid therapy followed by addition of cyclophosphamide or other glucocorticoid-sparing drugs.