Boston Criteria for cerebral amyloid angiopathy

Boston criteria for sporadic CAA version 2.0, 2022^[1]	Modified Boston criteria version 1.5, 2010^[2]	Boston criteria, 2001^[3]
Definite CAA
Full postmortem examination demonstrating: Spontaneous ICH, TFNE, cSAH, cognitive impairment Severe CAA with vasculopathy Absence of other diagnostic lesion	Full postmortem examination demonstrating: Lobar, cortical, or cortico-subcortical hemorrhage Severe CAA with vasculopathy Absence of other diagnostic lesion	Full postmortem examination demonstrating: Lobar, cortical, or cortico-subcortical hemorrhage Severe CAA with vasculopathy Absence of other diagnostic lesion
Probable CAA with supporting pathology
Clinical data and pathologic tissue (evacuated hematoma or cortical biopsy): Presentation with spontaneous ICH, TFNE, cSAH, or cognitive impairment Some degree of CAA in specimen Absence of other diagnostic lesion	Clinical data and pathologic tissue (evacuated hematoma or cortical biopsy): Lobar, cortical, or cortico-subcortical hemorrhage Some degree of CAA in specimen Absence of other diagnostic lesion	Clinical data and pathologic tissue (evacuated hematoma or cortical biopsy): Lobar, cortical, or cortico-subcortical hemorrhage Some degree of CAA in specimen Absence of other diagnostic lesion
Probable CAA
Clinical data and MRI demonstrating: Age ≥50 years Plus either: Presentation with spontaneous ICH, TFNE, or cognitive impairment Two or more lobar hemorrhagic lesions on T2-weighted imaging (eg, ICH, CMB, cSS, cSAH)^¶ Absence of other cause of hemorrhage^Δ or One lobar hemorrhagic lesion on T2-weighted imaging (eg, ICH, CMB, cSS, cSAH)^◊ One white matter feature (eg, >20 perivascular spaces in centrum semiovale or WMH in a multispot pattern) Absence of any deep hemorrhagic lesions on T2*-weighted imaging (eg, ICH, CMB)^¶ Absence of other cause of hemorrhagic lesions^Δ	Clinical data and MRI demonstrating: Age ≥55 years Plus either: Multiple hemorrhages restricted to lobar, cortical, or cortico-subcortical regions (cerebellar hemorrhage allowed) or Single lobar, cortical, or cortico-subcortical hemorrhage and focal^§ or disseminated^¥ superficial siderosis Absence of other cause of hemorrhage or superficial siderosis	Clinical data and MRI demonstrating: Age ≥55 years Multiple hemorrhages restricted to lobar, cortical, or cortico-subcortical regions (cerebellar hemorrhage allowed) Absence of other cause of hemorrhage^◊
Possible CAA
Clinical data and MRI demonstrating: Age ≥50 years Plus either: Presentation with spontaneous ICH, TFNE, cSAH, or cognitive impairment One lobar hemorrhagic lesion on T2-weighted imaging (eg, ICH, CMB, cSS, cSAH)^¶ Absence of other cause of hemorrhage^Δ or One white matter feature (eg, >20 perivascular spaces in centrum semiovale or WMH in a multispot pattern) Absence of any deep hemorrhagic lesions on T2-weighted imaging (eg, ICH, CMB)^¶ Absence of other cause of hemorrhagic lesions^Δ	Clinical data and MRI demonstrating: Age ≥55 years Plus either: Single lobar, cortical, or cortico-subcortical hemorrhage or Focal^§ or disseminated^¥ superficial siderosis Absence of other cause of hemorrhage or superficial siderosis	Clinical data and MRI demonstrating: Age ≥55 years Single lobar, cortical, or cortico-subcortical hemorrhage Absence of other cause of hemorrhage^◊

The Boston criteria incorporate clinical, radiologic, and pathologic data to provide diagnostic certainty for patients with suspected CAA. Successive iterations of these criteria reflect the evolving understanding of these diagnostic features. Refer to UpToDate topics for additional information on the diagnosis and evaluation of patients with suspected CAA.

CAA: cerebral amyloid angiopathy; ICH: intracerebral hemorrhage; TFNE: transient focal neurologic event; cSAH: convexity subarachnoid hemorrhage; MRI: magnetic resonance imaging; cSS: cortical superficial siderosis; WMH: white matter hyperintensity.

¶ Hemorrhagic lesion in the cerebellum is not counted as either lobar or deep hemorrhagic lesion.

Δ Other causes of hemorrhagic lesion: antecedent head trauma, hemorrhagic transformation of an ischemic stroke, arteriovenous malformation, hemorrhagic tumor, and vasculitis. Other causes of cortical superficial siderosis and acute convexity subarachnoid hemorrhage should also be excluded.

◊ Other causes of intracerebral hemorrhage: excessive warfarin (INR >3.0); antecedent head trauma or ischemic stroke; central nervous system tumor, vascular malformation, or vasculitis; and blood dyscrasia or coagulopathy. (INR >3.0 or other nonspecific laboratory abnormalities permitted for diagnosis of possible CAA.)

§ Siderosis restricted to 3 or fewer sulci.

¥ Siderosis affecting at least 4 sulci.

References:

Charidimou A, Boulouis G, Frosch MP, et al. The Boston criteria version 2.0 for cerebral amyloid angiopathy: A multicentre, retrospective, MRI-neuropathology diagnostic accuracy study. Lancet Neurol 2022; 21:714.
Linn J, Halpin A, Demaerel P, et al. Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology 2010; 74:1346.
Knudsen KA, Rosand J, Karluk D, Greenberg SM. Clinical diagnosis of cerebral amyloid angiopathy: Validation of the Boston criteria. Neurology 2001; 56:537.

Graphic 139328 Version 1.0

خرید پکیج

Boston Criteria for cerebral amyloid angiopathy