Sergi Martinez-Ramirez1, Jose-Rafael Romero2, Ashkan Shoamanesh3, Ann C McKee4, Ellis Van Etten5, Octavio Pontes-Neto5, Eric A Macklin6, Alison Ayres5, Eitan Auriel5, Jayandra J Himali7, Alexa S Beiser8, Charles DeCarli9, Thor D Stein10, Victor E Alvarez11, Matthew P Frosch12, Jonathan Rosand13, Steven M Greenberg5, M Edip Gurol5, Sudha Seshadri2, Anand Viswanathan5. 1. Neurology Department, J. Philip Kistler Stroke Research Center, Massachusetts General Hospital, Boston, MA, USA; Escola de Postgrau, Autonomous University of Barcelona, Barcelona, Spain. Electronic address: srmartinez@mgh.harvard.edu. 2. Department of Neurology, School of Medicine, Boston University, Boston, MA, USA; NHLBI's Framingham Heart Study, Framingham, MA, USA. 3. Neurology Department, J. Philip Kistler Stroke Research Center, Massachusetts General Hospital, Boston, MA, USA; NHLBI's Framingham Heart Study, Framingham, MA, USA. 4. Department of Neurology, School of Medicine, Boston University, Boston, MA, USA; NHLBI's Framingham Heart Study, Framingham, MA, USA; Department of Pathology, School of Medicine, Boston University, Boston, MA, USA; United States Department of Veterans Affairs, VA Boston Healthcare System, Boston, MA, USA. 5. Neurology Department, J. Philip Kistler Stroke Research Center, Massachusetts General Hospital, Boston, MA, USA. 6. Department of Biostatistics, Massachusetts General Hospital, Boston, MA, USA. 7. NHLBI's Framingham Heart Study, Framingham, MA, USA; Department of Biostatistics, School of Public Health, Boston University, Boston, MA, USA. 8. Department of Neurology, School of Medicine, Boston University, Boston, MA, USA; NHLBI's Framingham Heart Study, Framingham, MA, USA; Department of Biostatistics, School of Public Health, Boston University, Boston, MA, USA. 9. Department of Neurology, University of California-Davis, Sacramento, CA, USA. 10. Department of Pathology, School of Medicine, Boston University, Boston, MA, USA; United States Department of Veterans Affairs, VA Boston Healthcare System, Boston, MA, USA. 11. Department of Neurology, School of Medicine, Boston University, Boston, MA, USA; Center for the Study of Traumatic Encephalopathy, Boston University Alzheimer Disease Center, Boston, MA, USA. 12. Neuropathology Service, C.S. Kubik Laboratory for Neuropathology, Massachusetts General Hospital, Charlestown, MA, USA. 13. Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA, USA.
Abstract
INTRODUCTION: The Boston criteria are the basis for a noninvasive diagnosis of cerebral amyloid angiopathy (CAA) in the setting of lobar intracerebral hemorrhage (ICH). We assessed the accuracy of these criteria in individuals with lobar microbleeds (MBs) without ICH. METHODS: We identified individuals aged >55 years having brain magnetic resonance imaging (MRI) and pathological assessment of CAA in a single academic hospital and a community-based population (Framingham Heart Study [FHS]). We determined the positive predictive value (PPV) of the Boston criteria for CAA in both cohorts, using lobar MBs as the only hemorrhagic lesion to fulfill the criteria. RESULTS: We included 102 individuals: 55 from the hospital-based cohort and 47 from FHS (mean age at MRI 74.7 ± 8.5 and 83.4 ± 10.9 years; CAA prevalence 60% and 46.8%; cases with any lobar MB 49% and 21.3%; and cases with ≥2 strictly lobar MBs 29.1% and 8.5%, respectively). PPV of "probable CAA" (≥2 strictly lobar MBs) was 87.5% (95% confidence interval [CI], 60.4-97.8) and 25% (95% CI, 13.2-78) in hospital and general populations, respectively. DISCUSSION: Strictly lobar MBs strongly predict CAA in non-ICH individuals when found in a hospital context. However, their diagnostic accuracy in the general population appears limited.
INTRODUCTION: The Boston criteria are the basis for a noninvasive diagnosis of cerebral amyloid angiopathy (CAA) in the setting of lobar intracerebral hemorrhage (ICH). We assessed the accuracy of these criteria in individuals with lobar microbleeds (MBs) without ICH. METHODS: We identified individuals aged >55 years having brain magnetic resonance imaging (MRI) and pathological assessment of CAA in a single academic hospital and a community-based population (Framingham Heart Study [FHS]). We determined the positive predictive value (PPV) of the Boston criteria for CAA in both cohorts, using lobar MBs as the only hemorrhagic lesion to fulfill the criteria. RESULTS: We included 102 individuals: 55 from the hospital-based cohort and 47 from FHS (mean age at MRI 74.7 ± 8.5 and 83.4 ± 10.9 years; CAA prevalence 60% and 46.8%; cases with any lobar MB 49% and 21.3%; and cases with ≥2 strictly lobar MBs 29.1% and 8.5%, respectively). PPV of "probable CAA" (≥2 strictly lobar MBs) was 87.5% (95% confidence interval [CI], 60.4-97.8) and 25% (95% CI, 13.2-78) in hospital and general populations, respectively. DISCUSSION: Strictly lobar MBs strongly predict CAA in non-ICH individuals when found in a hospital context. However, their diagnostic accuracy in the general population appears limited.
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