BACKGROUND AND PURPOSE: Validation of the Boston criteria for the in vivo diagnosis of cerebral amyloid angiopathy (CAA) is challenging, because noninvasive diagnostic tests do not exist. Hereditary cerebral hemorrhage with amyloidosis-Dutch type is an accepted monogenetic model of CAA and diagnosis can be made with certainty based on DNA analysis. The aim of this study was to analyze and refine the existing Boston criteria in patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type. METHODS: We performed T2*-weighted MRI in 27 patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type to assess the presence and location of microbleeds, intracranial hemorrhages, and superficial siderosis. Using the Boston criteria, subjects were categorized as having: no hemorrhages, possible CAA, probable CAA, and hemorrhagic lesions not qualifying for CAA. The sensitivity of the Boston criteria was calculated separately using intracranial hemorrhages only and using intracranial hemorrhages and microbleeds. RESULTS: The sensitivity of the Boston criteria for probable CAA increased from 48% to 63% when microbleeds were included. For symptomatic subjects only, the sensitivity was 100%. No hemorrhages were identified in the deep white matter, basal ganglia, thalamus, or brainstem. Superficial siderosis, observed in 6 patients, did not increase the sensitivity of the Boston criteria in our study group. CONCLUSIONS: Our data show that using T2*-weighted MRI and including microbleeds increase the sensitivity of the Boston criteria. The exclusion of hemorrhages in the deep white matter, basal ganglia, thalamus, and brainstem does not lower the sensitivity of the Boston criteria.
BACKGROUND AND PURPOSE: Validation of the Boston criteria for the in vivo diagnosis of cerebral amyloid angiopathy (CAA) is challenging, because noninvasive diagnostic tests do not exist. Hereditary cerebral hemorrhage with amyloidosis-Dutch type is an accepted monogenetic model of CAA and diagnosis can be made with certainty based on DNA analysis. The aim of this study was to analyze and refine the existing Boston criteria in patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type. METHODS: We performed T2*-weighted MRI in 27 patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type to assess the presence and location of microbleeds, intracranial hemorrhages, and superficial siderosis. Using the Boston criteria, subjects were categorized as having: no hemorrhages, possible CAA, probable CAA, and hemorrhagic lesions not qualifying for CAA. The sensitivity of the Boston criteria was calculated separately using intracranial hemorrhages only and using intracranial hemorrhages and microbleeds. RESULTS: The sensitivity of the Boston criteria for probable CAA increased from 48% to 63% when microbleeds were included. For symptomatic subjects only, the sensitivity was 100%. No hemorrhages were identified in the deep white matter, basal ganglia, thalamus, or brainstem. Superficial siderosis, observed in 6 patients, did not increase the sensitivity of the Boston criteria in our study group. CONCLUSIONS: Our data show that using T2*-weighted MRI and including microbleeds increase the sensitivity of the Boston criteria. The exclusion of hemorrhages in the deep white matter, basal ganglia, thalamus, and brainstem does not lower the sensitivity of the Boston criteria.
Authors: Jonathan Graff-Radford; Timothy G Lesnick; Michelle M Mielke; Eleni Constantopoulos; Alejandro A Rabinstein; Scott A Przybelski; Prashanthi Vemuri; Hugo Botha; David T Jones; Vijay K Ramanan; Ronald C Petersen; David S Knopman; Bradley F Boeve; Melissa E Murray; Dennis W Dickson; Clifford R Jack; Kejal Kantarci; R Ross Reichard Journal: J Alzheimers Dis Date: 2021 Impact factor: 4.472
Authors: Aaron P Schultz; Reina W Kloet; Hamid R Sohrabi; Louise van der Weerd; Sanneke van Rooden; Marieke J H Wermer; Laure Grand Moursel; Maqsood Yaqub; Bart N M van Berckel; Pratishtha Chatterjee; Samantha L Gardener; Kevin Taddei; Anne M Fagan; Tammie L Benzinger; John C Morris; Reisa Sperling; Keith Johnson; Randall J Bateman; M Edip Gurol; Mark A van Buchem; Ralph Martins; Jasmeer P Chhatwal; Steven M Greenberg Journal: Ann Neurol Date: 2019-08-12 Impact factor: 10.422
Authors: Natalie S Ryan; António J Bastos-Leite; Jonathan D Rohrer; David J Werring; Nick C Fox; Martin N Rossor; Jonathan M Schott Journal: Brain Date: 2011-06-17 Impact factor: 13.501
Authors: Sergi Martinez-Ramirez; Jose-Rafael Romero; Ashkan Shoamanesh; Ann C McKee; Ellis Van Etten; Octavio Pontes-Neto; Eric A Macklin; Alison Ayres; Eitan Auriel; Jayandra J Himali; Alexa S Beiser; Charles DeCarli; Thor D Stein; Victor E Alvarez; Matthew P Frosch; Jonathan Rosand; Steven M Greenberg; M Edip Gurol; Sudha Seshadri; Anand Viswanathan Journal: Alzheimers Dement Date: 2015-06-13 Impact factor: 21.566
Authors: Andreas Charidimou; Zane Jaunmuktane; Jean-Claude Baron; Matthew Burnell; Pascale Varlet; Andre Peeters; John Xuereb; Rolf Jäger; Sebastian Brandner; David J Werring Journal: Neurology Date: 2013-11-27 Impact factor: 9.910