Childhood CNS vasculitis is an increasingly recognized inflammatory brain disease, which may develop as a primary condition, or secondary to an underlying systemic illness. Primary angiitis of the central nervous system of childhood (cPACNS) is one of the leading causes of severe, non-accidental brain injury including acute ischemic stroke, refractory status epilepticus, psychosis, and progressive cognitive decline in previously healthy children. Clinical recognition and rapid diagnostic evaluation are crucial to prevent brain damage. The differential diagnosis has to be thoroughly explored prior to invasive testing including distinct metabolic conditions, non-inflammatory vasculopathies and non-vasculitic, inflammatory brain diseases. Biomarkers are available for many conditions. Angiography is a key imaging modality for large vessel cPACNS. Angiography-negative small-vessel CNS vasculitis mandates invasive brain biopsies to confirm the diagnosis. Recognition, rapid diagnostic evaluation and initiation of targeted therapies are mandatory to control inflammation and prevent severe neurological impairment in children. The presentation will summarizes the current information on diagnosis and differential diagnosis, diagnostic algorithms and treatment efficacy and safety in childhood CNS vasculitis.
Disclosure of Interest None Declared