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Cerebral amyloid angiopathy and CNS vasculitis 

Cerebral amyloid angiopathy and CNS vasculitis
Cerebral amyloid angiopathy and CNS vasculitis

Sergi Martinez-Ramirez

, Steven M. Greenberg

, and Anand Viswanathan

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date: 21 October 2021

This chapter focuses on two important groups of brain pathologies: cerebral amyloid angiopathy (CAA) and central nervous system vasculitis (CNS vasculitis). CAA is a heterogeneous group of entities characterized by the deposition of amyloid proteins in the vessel walls of small-sized arteries and capillaries of leptomeninges and cerebral cortex. The accumulation of amyloid in brain vessels underlies most lobar intracerebral haemorrhages (ICH) in the elderly, but CAA’s clinical spectrum also includes cerebral ischaemia and cognitive decline. The term CNS vasculitis refers generically to the inflammation of cerebral blood vessels. A wide and heterogeneous group of diseases may cause CNS vascular inflammation, either primarily or secondarily. Primary CNS vasculities are largely represented by two entities: CAA-related inflammation (CAA-RI) and primary angiitis of the CNS (PACS). CAA-RI is particularly interesting as it establishes a unique link between a protein deposition-based disease (CAA) and a primary inflammatory process of the CNS.

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