Article Text

other Versions

PDF
Review
Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum
  1. Andreas Charidimou,
  2. Qiang Gang,
  3. David J Werring
  1. Stroke Research Group, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology and the National Hospital for Neurology and Neurosurgery, Queen Square, London, UK
  1. Correspondence to Dr D J Werring, National Hospital for Neurology and Neurosurgery, Box 6, Queen Square, London WC1N 3BG, UK; d.werring{at}ucl.ac.uk

Abstract

Sporadic cerebral amyloid angiopathy (CAA) is a common age related cerebral small vessel disease, characterised by progressive deposition of amyloid-β (Aβ) in the wall of small to medium sized arteries, arterioles and capillaries of the cerebral cortex and overlying leptomeninges. Previously considered to be a rare neurological curiosity, CAA is now recognised as an important cause of spontaneous intracerebral haemorrhage and cognitive impairment in the elderly, two fundamental challenges in the field of cerebrovascular disease. Our understanding of the pathophysiology and clinical manifestations of CAA continues to evolve rapidly, with the use of transgenic mouse models and advanced structural and/or molecular neuroimaging techniques. Yet, despite remarkable recent interest, CAA remains under-recognised by neurologists and stroke physicians. In this review, a fresh look at key developments in understanding the complex pathophysiology, important clinical and radiological features, diagnostic approaches and prospects for rational therapies for this enigmatic small vessel disorder is provided.

Statistics from Altmetric.com

Footnotes

  • Funding AC receives research support from the Greek State Scholarship Foundation. DJW receives research support from the Department of Health/Higher Education Funding Council for England (Clinical Senior Lectureship Award) and the Stroke Association. This work was undertaken at UCLH/UCL who received a proportion of funding from the Department of Health's NIHR Biomedical Research Centres funding scheme.

  • Competing interests None.

  • Provenance and peer review Commissioned; externally peer reviewed.

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.