Difference between revisions of "Cerebral amyloid angiopathy"

 
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** CAA is prevalent in 20-40% of non-demented, and 50-60% of demented elderly (age range 80-90 years) (Keage et al, 2009)
 
** CAA is prevalent in 20-40% of non-demented, and 50-60% of demented elderly (age range 80-90 years) (Keage et al, 2009)
 
** in Alzheimer disease it is prevalent in 82-98% of cases (Jellinger 2002)
 
** in Alzheimer disease it is prevalent in 82-98% of cases (Jellinger 2002)
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 +
 
*Clinical presentation
 
*Clinical presentation
 
**spontaneous lobar ICH, cognitive impairment, dementia
 
**spontaneous lobar ICH, cognitive impairment, dementia
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***moderate to severe CAA in older community dwelling population was associated with lower perceptual speed and episodic memory (Arvanitakis et al 2011)
 
***moderate to severe CAA in older community dwelling population was associated with lower perceptual speed and episodic memory (Arvanitakis et al 2011)
 
**"amyloid spells", or transient focal neurological episodes (TFNEs), generally associated with SAH or superficial siderosis
 
**"amyloid spells", or transient focal neurological episodes (TFNEs), generally associated with SAH or superficial siderosis
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 +
 
*Diagnostics
 
*Diagnostics
 
**MRI with blood sensitive sequences
 
**MRI with blood sensitive sequences
 
***multiple lobar cerebral microbleeds, cortical superficial siderosis, white matter disease, cortical microinfarcts, MRI-visible perivascular spaces in the centrum semiovale
 
***multiple lobar cerebral microbleeds, cortical superficial siderosis, white matter disease, cortical microinfarcts, MRI-visible perivascular spaces in the centrum semiovale
***Modified '''Boston Criteria''' for Cerebral Amyloid Angiopathy
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***Modified '''Boston Criteria''' for Cerebral Amyloid Angiopathy (Linn et al, 2010)
 
****Definite CAA - full post-mortem examination demonstrating:
 
****Definite CAA - full post-mortem examination demonstrating:
 
*****lobar, cortical, or cortical-subcortical hemorrhage
 
*****lobar, cortical, or cortical-subcortical hemorrhage
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Keage HA, et al. Population studies of sporadic cerebral amyloid angiopathy and dementia: a systematic review. BMC Neurol (2009). https://www-ncbi-nlm-nih-gov/19144113
 
Keage HA, et al. Population studies of sporadic cerebral amyloid angiopathy and dementia: a systematic review. BMC Neurol (2009). https://www-ncbi-nlm-nih-gov/19144113
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Linn J, et al. Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology. 74:1346–1350. (2010). https://www-ncbi-nlm-nih-gov/20421578

Latest revision as of 13:21, 17 April 2022

Cerebral amyloid angiopathy (CAA) is the cerebrovascular deposition of amyloid-beta. It is common amongst elderly, a cause of spontaneous intracerebral hemorrhage, and an important contributor to age-related cognitive decline (Charidimou et al 2017).

  • Epidemiology
    • CAA is prevalent in 20-40% of non-demented, and 50-60% of demented elderly (age range 80-90 years) (Keage et al, 2009)
    • in Alzheimer disease it is prevalent in 82-98% of cases (Jellinger 2002)


  • Clinical presentation
    • spontaneous lobar ICH, cognitive impairment, dementia
    • cognitive decline: the independent contribution of CAA is difficult to determine because of the high co-existance of Alzheimer's disease and other age-related pathologies, though it appears to independently contribute to cognitive decline beyond its accompanying pathologies (Charidimou et al 2017).
      • moderate to severe CAA in older community dwelling population was associated with lower perceptual speed and episodic memory (Arvanitakis et al 2011)
    • "amyloid spells", or transient focal neurological episodes (TFNEs), generally associated with SAH or superficial siderosis


  • Diagnostics
    • MRI with blood sensitive sequences
      • multiple lobar cerebral microbleeds, cortical superficial siderosis, white matter disease, cortical microinfarcts, MRI-visible perivascular spaces in the centrum semiovale
      • Modified Boston Criteria for Cerebral Amyloid Angiopathy (Linn et al, 2010)
        • Definite CAA - full post-mortem examination demonstrating:
          • lobar, cortical, or cortical-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) demonstrating:
          • lobar, cortical, or cortical-subcortical hemorrhage (ICH, CMB, or cSS)
          • some degree of CAA in specimen
          • absence of other diagnostic lesion
        • Probable CAA - clinical data and MRI or CT demonstrating:
          • multiple hemorrhages (ICH, CMB) restricted to lobar, cortical, or cortical-subcortical regions (cerebellar hemorrhage allowed), OR single lobar, cortical, or cortical-sbcortical hemorrhage and cSS (focal or disseminated)
          • age 55 or older
          • absence of other causes of hemorrhage
        • Possible CAA - clinical data and MRI or CT demonstrating:
          • single lobar, cortical, or cortical-subcortical ICH, CMB, or cSS (focal or disseminated)
          • age 55 or older
          • absence of other cause of hemorrhage


References

Arvanitakis Z et al. Cerebral amyloid angiopathy pathology and cognitive domains in older persons. Ann Neurol (2011). https://www-ncbi-nlm-nih-gov/pubmed/21387377

Charidimou A, et al. Emerging concepts in sporadic cerebral amyloid angiopathy. Brain. Jul 1;140(7):1829-1850. (2017) https://pubmed.ncbi.nlm.nih.gov/28334869/

Jellinger KA. Alzheimer disease and cerebrovascular pathology: an update. J Neural Transm (Vienna). May;109(5-6):813-36 (2002). https://pubmed-ncbi-nlm-nih-gov/12111471/

Keage HA, et al. Population studies of sporadic cerebral amyloid angiopathy and dementia: a systematic review. BMC Neurol (2009). https://www-ncbi-nlm-nih-gov/19144113

Linn J, et al. Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology. 74:1346–1350. (2010). https://www-ncbi-nlm-nih-gov/20421578