The most frequent manifestation of Cerebral Amyloid Angiopathy (CAA) is symptomatic, spontaneous intracerebral haemorrhage (ICH), preferentially affecting lobar regions.

CAA represents the second most common cause of ICH after hypertensive angiopathy in the general population, and it is the most frequent cause of cerebral haemorrhage in the elderly. Moreover, ischaemic stroke, transient ischemic attacks, subarachnoid haemorrhage, and progressive cognitive decline are also common events and clinical manifestations.

The characteristic pathological lesion of CAA is the accumulation of amyloid proteins in the leptomeningeal vessels and cortical arterioles. This phenomenon leads to vessel stiffening and a subsequent rupture of the vessel walls. It is responsible for cerebrovascular diseases, in particular lobar haemorrhages, microbleeds, and cortical siderosis.

The inflammatory form of CAA, also known as Cerebral Amyloid Angiopathy-Related Inflammation (CAARI), has recently been described. This form of potentially reversible encephalopathy is characterized by neurobehavioral symptoms, seizures, and stroke-like signs, in contrast to the classical signs of CAA, and neuroimaging shows typical findings in this condition. There are two pathological variants of CAARI: the first with predominant perivascular inflammatory infiltrates, properly called CAARI, and the second characterized by a transmural vasculitic process with or without granuloma formation, A-beta-related angitis. The inflammatory component of this syndrome could be treated with immune modulating therapies and this makes the diagnosis of CAARI very important.

The diagnosis and, in particular, the management of patients affected by CAA or CAARI is often challenging.

This Special Issue targets any innovative research that provides us with a better understanding of the pathogenetic, clinical, and management aspects of CAA.

Prof. Dr. Raffaele Nardone
Dr. Federico Pacei
Guest Editors

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• Cerebral Amyloid Angiopathy
• Cerebral Amyloid Angiopathy Related Inflammation
• cortical siderosis
• cerebral hemorrhage
• stroke
• microbleeds
• cognitive decline