RT Journal Article
T1 The Boston criteria version 2.0 for cerebral amyloid angiopathy: a multicentre, retrospective, MRI-neuropathology diagnostic accuracy study.
A1 Charidimou, Andreas
A1 Boulouis, Gregoire
A1 Frosch, Matthew P
A1 Baron, Jean-Claude
A1 Pasi, Marco
A1 Albucher, Jean Francois
A1 Banerjee, Gargi
A1 Barbato, Carmen
A1 Bonneville, Fabrice
A1 Brandner, Sebastian
A1 Calviere, Lionel
A1 Caparros, François
A1 Casolla, Barbara
A1 Cordonnier, Charlotte
A1 Delisle, Marie-Bernadette
A1 Deramecourt, Vincent
A1 Dichgans, Martin
A1 Gokcal, Elif
A1 Herms, Jochen
A1 Hernandez-Guillamon, Mar
A1 Jäger, Hans Rolf
A1 Jaunmuktane, Zane
A1 Linn, Jennifer
A1 Martinez-Ramirez, Sergi
A1 Martínez-Sáez, Elena
A1 Mawrin, Christian
A1 Montaner, Joan
A1 Moulin, Solene
A1 Olivot, Jean-Marc
A1 Piazza, Fabrizio
A1 Puy, Laurent
A1 Raposo, Nicolas
A1 Rodrigues, Mark A
A1 Roeber, Sigrun
A1 Romero, Jose Rafael
A1 Samarasekera, Neshika
A1 Schneider, Julie A
A1 Schreiber, Stefanie
A1 Schreiber, Frank
A1 Schwall, Corentin
A1 Smith, Colin
A1 Szalardy, Levente
A1 Varlet, Pascale
A1 Viguier, Alain
A1 Wardlaw, Joanna M
A1 Warren, Andrew
A1 Wollenweber, Frank A
A1 Zedde, Marialuisa
A1 van Buchem, Mark A
A1 Gurol, M Edip
A1 Viswanathan, Anand
A1 Al-Shahi Salman, Rustam
A1 Smith, Eric E
A1 Werring, David J
A1 Greenberg, Steven M
AB Cerebral amyloid angiopathy (CAA) is an age-related small vessel disease, characterised pathologically by progressive deposition of amyloid β in the cerebrovascular wall. The Boston criteria are used worldwide for the in-vivo diagnosis of CAA but have not been updated since 2010, before the emergence of additional MRI markers. We report an international collaborative study aiming to update and externally validate the Boston diagnostic criteria across the full spectrum of clinical CAA presentations. In this multicentre, hospital-based, retrospective, MRI and neuropathology diagnostic accuracy study, we did a retrospective analysis of clinical, radiological, and histopathological data available to sites participating in the International CAA Association to formulate updated Boston criteria and establish their diagnostic accuracy across different populations and clinical presentations. Ten North American and European academic medical centres identified patients aged 50 years and older with potential CAA-related clinical presentations (ie, spontaneous intracerebral haemorrhage, cognitive impairment, or transient focal neurological episodes), available brain MRI, and histopathological assessment for CAA diagnosis. MRI scans were centrally rated at Massachusetts General Hospital (Boston, MA, USA) for haemorrhagic and non-haemorrhagic CAA markers, and brain tissue samples were rated by neuropathologists at the contributing sites. We derived the Boston criteria version 2.0 (v2.0) by selecting MRI features to optimise diagnostic specificity and sensitivity in a prespecified derivation cohort (Boston cases 1994-2012, n=159), then externally validated the criteria in a prespecified temporal validation cohort (Boston cases 2012-18, n=59) and a geographical validation cohort (non-Boston cases 2004-18; n=123), comparing accuracy of the new criteria to the currently used modified Boston criteria with histopathological assessment of CAA as the diagnostic standard. We also assessed performance of the v2.0 criteria in patients across all cohorts who had the diagnostic gold standard of brain autopsy. The study protocol was finalised on Jan 15, 2017, patient identification was completed on Dec 31, 2018, and imaging analyses were completed on Sept 30, 2019. Of 401 potentially eligible patients presenting to Massachusetts General Hospital, 218 were eligible to be included in the analysis; of 160 patient datasets from other centres, 123 were included. Using the derivation cohort, we derived provisional criteria for probable CAA requiring the presence of at least two strictly lobar haemorrhagic lesions (ie, intracerebral haemorrhages, cerebral microbleeds, or foci of cortical superficial siderosis) or at least one strictly lobar haemorrhagic lesion and at least one white matter characteristic (ie, severe visible perivascular spaces in centrum semiovale or white matter hyperintensities in a multispot pattern). The sensitivity and specificity of these criteria were 74·8% (95% CI 65·4-82·7) and 84·6% (71·9-93·1) in the derivation cohort, 92·5% (79·6-98·4) and 89·5% (66·9-98·7) in the temporal validation cohort, 80·2% (70·8-87·6) and 81·5% (61·9-93·7) in the geographical validation cohort, and 74·5% (65·4-82·4) and 95·0% (83·1-99·4) in all patients who had autopsy as the diagnostic standard. The area under the receiver operating characteristic curve (AUC) was 0·797 (0·732-0·861) in the derivation cohort, 0·910 (0·828-0·992) in the temporal validation cohort, 0·808 (0·724-0·893) in the geographical validation cohort, and 0·848 (0·794-0·901) in patients who had autopsy as the diagnostic standard. The v2.0 Boston criteria for probable CAA had superior accuracy to the current Boston criteria (sensitivity 64·5% [54·9-73·4]; specificity 95·0% [83·1-99·4]; AUC 0·798 [0·741-0854]; p=0·0005 for comparison of AUC) across all individuals who had autopsy as the diagnostic standard. The 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. US National Institutes of Health (R01 AG26484).
YR 2022
FD 2022
LK http://hdl.handle.net/10668/22556
UL http://hdl.handle.net/10668/22556
LA en
DS RISalud
RD Apr 19, 2025