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Criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation (CAA-ri)

Criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation (CAA-ri)
Diagnosis Criteria
Probable CAA-ri
  1. Age ≥40 years
  2. Presence of one or more of the following clinical features: headache, decrease in consciousness, behavioral change, or focal neurological signs and seizures; the presentation is not directly attributable to an acute ICH
  3. MRI shows unifocal or multifocal WMH lesions (corticosubcortical or deep) that are asymmetric and extend to the immediately subcortical white matter; the asymmetry is not due to past ICH
  4. Presence of one or more of the following corticosubcortical hemorrhagic lesions: cerebral macrobleed, cerebral microbleed, or cortical superficial siderosis[1]
  5. Absence of neoplastic, infectious, or other cause
Possible CAA-ri
  1. Age ≥40 years
  2. Presence of one or more of the following clinical features: headache, decrease in consciousness, behavioral change, or focal neurological signs and seizures; the presentation is not directly attributable to an acute ICH
  3. MRI shows WMH lesions that extend to the immediately subcortical white matter
  4. Presence of one or more of the following corticosubcortical hemorrhagic lesions: cerebral macrobleed, cerebral microbleed, or cortical superficial siderosis[1]
  5. Absence of neoplastic, infectious, or other cause
CAA-ri: cerebral amyloid angiopathy-related inflammation; ICH: intracerebral hemorrhage; MRI: magnetic resonance imaging; WMH: white matter hyperintensity.
Reference:
  1. Charidimou A, Linn J, Vernooij MW, et al. Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. Brain 2015; 138:2126.
Reproduced with permission from: Auriel E, Charidimou A, Gurol ME, et al. Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation. JAMA Neurol 2016; 73(2):197-202. Copyright © 2016 American Medical Association. All rights reserved.
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