CNS vasculitis in children

Inflammatory brain diseases in childhood are underrecognized and lead to life-threatening neurological deficits. Early recognition and diagnosis of inflammatory brain diseases is critical, as the reversibility of the neurological deficits is closely related to early initiation of treatment and prevention of secondary brain tissue damage. Primary childhood CNS vasculitis is the most common cause of inflammatory brain disease in childhood. Clinical features, laboratory tests and imaging can be non-conclusive and overlap with other inflammatory brain diseases, such as demyelinating diseases. This review focuses on recent publications on epidemiology, pathogenesis, and treatment in childhood CNS vasculitis and relevant publications from the rapidly expanding differential diagnosis for the subtypes of CNS vasculitis, particularly the demyelinating brain diseases.