Cerebral amyloid angiopathy: one single entity?

Current Opinion in Neurology, vol. 38, pp. 29–34

Abstract

PURPOSE OF REVIEW: Cerebral amyloid angiopathy (CAA) is a common brain disorder among the elderly and individuals with Alzheimer's disease, where accumulation of amyloid-ß can lead to intracerebral hemorrhage and dementia. This review discusses recent developments in understanding the pathophysiology and phenotypes of CAA. RECENT FINDINGS: CAA has a long preclinical phase starting decades before symptoms emerge. Its pathophysiology follows consecutive stages of amyloid-ß deposition, decreased vascular reactivity, nonhemorrhagic changes, and ultimately hemorrhages. Although impaired perivascular clearance is the leading hypothesis underlying CAA, several lines of evidence suggest that glymphatic dysfunction also plays a significant role in the disease process. Despite its common pathway, the disease course is variable. Some patients develop more microbleeds, while others develop larger hemorrhages, suggesting a differentiation in vascular remodeling. Some patients with CAA develop a symptomatic immune response, and inflammation could be an important contributor to vascular damage in CAA in general. Furthermore, the prion-like transmission of amyloid-β has been identified as a cause of iatrogenic CAA occurring decades after neurosurgical procedures involving cadaveric dura mater. SUMMARY: Emerging evidence of sporadic, hereditary, inflammatory, and iatrogenic CAA suggests a complex interplay between brain clearance, inflammation and vascular remodeling leading to a diverse clinical phenotype.

Authors 2

1. Emma A. Koemans Aachen Department of Neurology, Leiden University Medical Center (LUMC)

   Leiden University Medical Center

   Affiliation as printed

   Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands

2. Ellis S. van Etten corresponding Aachen Department of Neurology, Leiden University Medical Center (LUMC) to Ellis S. van Etten

   Leiden University Medical Center

   Affiliation as printed

   Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands

   to Ellis S. van Etten, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands. Tel: +31 715 262 257; e-mail: [email protected]

   e-mail: [email protected]

   to Ellis S. van Etten, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands. Tel: +31 715 262 257