Boston criteria 2.0 for cerebral amyloid angiopathy

Last revised by Rohit Sharma on 23 Feb 2024

The Boston criteria 2.0 were proposed in 2022 in order to better include leptomeningeal and white matter characteristics into the diagnoses of probable and possible cerebral amyloid angiopathy (CAA) 1. They consist of combined clinical, imaging and pathological parameters, and are based upon the original Boston criteria and modified Boston criteria, which were proposed in 1995 and 2010 respectively 2,3

The criteria are divided into four tiers 1:

  • definite CAA

  • probable CAA with supporting pathology

    • clinical data and pathological tissue (evacuated hematoma or cortical biopsy) demonstrating:

  • probable CAA

    • pathological confirmation not required

    • for patients aged 50 years and older

    • presentation with spontaneous intracerebral hemorrhage, transient focal neurological episodes, or cognitive impairment or dementia

    • MRI criteria:

  • possible CAA

In its initial publication, the Boston criteria 2.0 for diagnosis of 'probable CAA' was validated against multiple different cohorts, and against patients who had had autopsy it had a sensitivity of 74.5% (95% confidence interval (CI) 65.4% to 82.4%) and specificity of 95% (95% CI 83.1% to 99.4%), which is superior compared to when the modified Boston criteria were applied to the same patient cohorts 1.

* "Other cause of hemorrhagic lesions" are: antecedent head trauma, hemorrhagic transformation of an ischemic stroke, arteriovenous malformation, hemorrhagic tumor, warfarin therapy with INR >3, and vasculitis 1.

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