Cerebral amyloid angiopathy (CAA)

Created 13/04/2021, last revision 23/01/2023

  • a heterogeneous group of sporadic or familial diseases characterized by amyloid deposits in the walls of small and medium-sized cerebral vessels
  • mainly affects older people, rare familial forms (Icelandic and Dutch type) occur in younger age
  • CAA is responsible for ~2-10% of primary intracranial hemorrhages (30-70% of lobar hematomas in elderly patients) [Chao, 2006]


  • amyloid deposits in small and medium-sized cerebral arteries without systemic amyloidosis
  • there is some association with typical Alzheimer’s changes, such as neuritic plaques and neurofibrillary tangles
Cerebral amyloid angiopathy

Clinical presentation

  • Transient Focal Neurological Episodes (TFNE), also called “amyloid spells
  • recurrent lobar intracerebral hemorrhages (ICH)
  • convexial SAH
  • hemocephalus
  • arteriolopathy with leukoaraiosis and encephalopathy
  • development of vascular dementia at an advanced stage of the disease
  • transient positive and negative symptoms (also “amyloid spells”)
    • positive symptoms – “aura-like” spreading paresis, visual phenomena (monocular blurred vision, flashes, teichopsia), twitching of limbs
    • negative symptoms – transient focal symptoms – paresis, speech and visual disturbances
  • positive symptoms predominate
  • usually, there are multiple stereotyped episodes, typically lasting 10-30 minutes
  • occurrs in about 14% of CAA patients [Charidimou, 2012]
  • very often caused by convexial SAH
    • FLAIR, DWI/ADC, and GRE/SWI are optimal for diagnostic workup
    • frequently, convexial SAH is seen along with microbleeds or parenchymal hematoma, but also recent lesions on DWI –  etiology is heterogeneous
  • may be confused with TIA (antithrombotic drugs further increase the risk of ICH) or stroke (high risk of ICH during thrombolysis)
  • TFNE is often followed by subsequent ICH (37.5% / 2 months)!! [Charidimou, 2012]

Diagnostic evaluation

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Anticoagulation in patients with suspected CAA

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Thrombolysis in patients with suspected CAA

  • in a patient with CMBs < 10, IVT is possible (AHA/ASA 2019 IIa/B-NR)
  • with CMBs >10, IVT is associated with a higher risk of ICH; the expected benefit of treatment must outweigh the risk (AHA/ASA 2019 IIb/B-NR)
    • consider IVT in a severe deficit in a patient without a severe premorbid condition

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Cerebral amyloid angiopathy (CAA)
link: https://www.stroke-manual.com/cerebral-amyloid-angiopathy-caa/