SEARCHNARRATIVE_REVIEWneurology, preclinical research

narrative_review·neurology, preclinical research, biomedical research, systematic review·PMC10545523

Clinical considerations in early-onset cerebral amyloid angiopathy

Brain · 8 authors, 12 centres

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TL;DR

This narrative review describes the genetic and iatrogenic causes of early-onset cerebral amyloid angiopathy (CAA) and provides a structured approach for investigation and management.

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This narrative review synthesizes evidence on early-onset cerebral amyloid angiopathy (CAA), a rare condition with genetic or iatrogenic causes. The review proposes a structured investigation approach and discusses management considerations.

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narrative_review

SPECIALTY

neurology

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AI pipeline

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ORIGINAL PAPER

PMC10545523

Clinical considerations in early-onset cerebral amyloid angiopathy

Brain

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ABSTRACT

Abstract Cerebral amyloid angiopathy (CAA) is an important cerebral small vessel disease associated with brain haemorrhage and cognitive change. The commonest form, sporadic amyloid-β CAA, usually affects people in mid- to later life. However, early-onset forms, though uncommon, are increasingly recognized and may result from genetic or iatrogenic causes that warrant specific and focused investigation and management. In this review, we firstly describe the causes of early-onset CAA, including monogenic causes of amyloid-β CAA ( APP missense mutations and copy number variants; mutations of PSEN1 and PSEN2 ) and non-amyloid-β CAA (associated with ITM2B , CST3 , GSN , PRNP and TTR mutations), and other unusual sporadic and acquired causes including the newly-recognized iatrogenic subtype. We then provide a structured approach for investigating early-onset CAA, and highlight important management considerations. Improving awareness of these unusual forms of CAA amongst healthcare professionals is essential for facilitating their prompt diagnosis, and an understanding of their underlying pathophysiology may have implications for more common, late-onset, forms of the disease.

CITATION EXPORT

Gargi Banerjee, John Collinge, Nick C Fox et al.. Clinical considerations in early-onset cerebral amyloid angiopathy. Brain. (2023). https://doi.org/10.1093/brain/awad193

AUTHORS

8 · 12 CENTRES

Gargi Banerjee, John Collinge, Nick C Fox, Tammaryn Lashley, Simon Mead, Jonathan M Schott, David J Werring, Natalie S Ryan

MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, W1W 7FF, UK
MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, W1W 7FF, UK
Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK
UK Dementia Research Institute at UCL, London, WC1E 6BT, UK
The Queen Square Brain Bank for Neurological Disorders, Department of Clinical and Movement Disorders, UCL Queen Square Institute of Neurology, London, W1 1PJ, UK
Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK
MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, W1W 7FF, UK
Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK
UK Dementia Research Institute at UCL, London, WC1E 6BT, UK
Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK
Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK
UK Dementia Research Institute at UCL, London, WC1E 6BT, UK