Inflammatory Cerebral Amyloid Angiopathy, Amyloid-β–Related Angiitis, and Primary Angiitis of the Central Nervous System

Inflammatory Cerebral Amyloid Angiopathy, Amyloid-β-Related Angiitis, and Primary Angiitis of the Central Nervous System: Similarities and Differences.

C erebral amyloid angiopathy (CAA) results from depo-sition of amyloid-β fibrils in the wall of the small and medium-sized blood vessels, mostly arteries of the lepto-meninges and cerebral cortex. Architectural disruption of amyloid-β laden vessels, occasionally with fibrinoid necrosis, leads to perivascular leakage. Vascular rupture causes lobar microbleeds or hematomas and high-convexity suba...

Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy.

Idiopathic or primary angiitis of the CNS (PACNS) and cerebral amyloid angiopathy (CAA) are unusual vasculopathies generally regarded as unrelated disorders. A few case reports have, however, described granulomatous angiitis in patients with sporadic, amyloid beta peptide (Abeta)-related CAA. Here we describe the clinical, neuroradiological and neuropathological features of nine patients with A...

Imaging Findings of Cerebral Amyloid Angiopathy, Aβ-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy–Related Inflammation

Vascular inflammation is present in a subset of patients with cerebral amyloid angiopathy (CAA) and has a major influence in determining the disease manifestations. Radiological characterization of this subset is particularly important to achieve early recognition and treatment. We conducted this study to investigate the role of imaging in differentiating CAA with and without inflammation. We r...

primary angiitis of the central nervous system

primary angiitis of the central nervous system (pacns) is an idiopathic disorder (vasculitis) restricted to the central nervous system (cns). it often presents with focal neurological deficits suggesting stroke or a combination of confusion and headache. we herein report three cases with various combinations of fever, partial seizure, encephalopathy, paresis, headache and ataxia. one of them wa...

Primary Angiitis of the Central Nervous System