Cerebral amyloid angiopathy criteria: the next generation

Abstract

For many years, cerebral amyloid angiopathy (CAA), which is an important cause of intracerebral haemorrhage in older people, was diagnosed on the basis of pathology, by the presence of amyloid-β protein deposition in small and medium arteries and capillaries within the cortex and leptomeninges. Publication of the Boston criteria for CAA allowed an imaging-based clinical diagnosis of probable CAA to be made without the need for brain tissue. 1 Greenberg SM Briggs ME Hyman BT et al. Apolipoprotein E epsilon 4 is associated with the presence and earlier onset of hemorrhage in cerebral amyloid angiopathy. Stroke. 1996; 27: 1333-1337 Crossref PubMed Scopus (216) Google Scholar As knowledge in the field accrued and imaging techniques improved, the criteria were updated in 2010 (modified Boston criteria) 2 Leurent C Goodman JA Zhang Y et al. Immunotherapy with ponezumab for probable cerebral amyloid angiopathy. Ann Clin Transl Neurol. 2019; 6: 795-806 Crossref PubMed Scopus (26) Google Scholar to allow inclusion of cortical superficial siderosis as an additional haemorrhagic lesion, which improved their sensitivity. The Boston criteria have been influential in research (by increasing interest in CAA internationally and providing the diagnostic framework for clinical trials) and useful in clinical practice (by aiding clinicians and patients to make better informed decisions, for example for patients who have atrial fibrillation and meet criteria for possible or probable CAA, and so have an increased risk of intracerebral haemorrhage). 3 DeSimone CV Graff-Radford J El-Harasis MA Rabinstein AA Asirvatham SJ Holmes Jr, DR Cerebral amyloid angiopathy: diagnosis, clinical implications, and management strategies in atrial fibrillation. J Am Coll Cardiol. 2017; 70: 1173-1182 Crossref PubMed Scopus (50) Google Scholar The Boston criteria version 2.0 for cerebral amyloid angiopathy: a multicentre, retrospective, MRI–neuropathology diagnostic accuracy studyThe Boston criteria v2.0 incorporate emerging MRI markers of CAA to enhance sensitivity without compromising their specificity in our cohorts of patients aged 50 years and older presenting with spontaneous intracerebral haemorrhage, cognitive impairment, or transient focal neurological episodes. Future studies will be needed to determine generalisability of the v.2.0 criteria across the full range of patients and clinical presentations. Full-Text PDF