Vasculitis of the Central Nervous System

Central nervous system (CNS) vasculitides are defined by inflammatory changes of intracranial and, occasionally, spinal vessel walls. They occur as cerebral manifestations of systemic vasculitides or can be a result of non-vasculitic diseases or present as isolated angiitis of the CNS. Although being rare diseases, they frequently have to be considered in the differential diagnosis due to the etiologically non-specific nature of clinical and radiological findings. A subacute presentation of multifocal neurological symptoms, headache, psychiatric symptoms or systemic inflammation, or the occurrence of stroke in patients of young age lacking a vascular risk profile or with known concomitant rheumatological diseases should raise the suspicion of CNS vasculitis. In addition to the confirmation of vasculitis, its etiology must be clarified, and - most importantly - infections have to be ruled out. In the case of isolated CNS angiitis, even invasive diagnostic measures such as cerebral angiography and CNS biopsy, which is the diagnostic gold standard, often do not lead to a definitive diagnosis. In addition to the treatment of any causative conditions, cerebral vasculitides are typically treated with a combination of corticosteroids and immunosuppressants. Instead of a uniform approach, disease severity and the degree of diagnostic certainty should be considered for the choice of treatment.