Primary angiitis of the CNS (PACNS) and Behçet disease

Abstract

Abstract Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache. This guideline was developed in order to support clinicians in the diagnosis and treatment of primary angiitis of the CNS (PACNS) and Neuro-Behçet. It is based on a medline research and was developed in a modified Delphi process and approved by the involved societies. This article is an abridged and translated version of the guideline published in DGNeurologie: Berlit, P. & Krämer, M. DGNeurologie (2018) 1: 17. https://doi.org/10.1007/s42451-018-0001-y

Keywords

• Primary angiitis of the CNS (PACNS)
• Reversible cerebral vasoconstriction syndrome (RCVS)
• Behçet disease
• Neuro-Behçet
• Isolated angiitis of the CNS