J Neurol Neurosurg Psychiatry 83:124-137 doi:10.1136/jnnp-2011-301308
  • Cerebrovascular disease
  • Review

Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum

Editor's Choice
  1. 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}
  1. Contributors AC and DJW designed the draft paper. AC and QG performed the bibliographic search; DJW reviewed the literature included in the paper. All authors were involved in drafting the paper. DJW and AC revised the draft paper. AC designed the artwork with input and revisions by DJW.

  • Received 30 August 2011
  • Accepted 2 October 2011
  • Published Online First 5 November 2011


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.


  • 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.

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