What are uncommon presentations of cerebral amyloid angiopathy (CAA)?

Updated: Dec 19, 2018
  • Author: Ravi S Menon, MD; Chief Editor: Helmi L Lutsep, MD  more...
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Answer

Answer

Uncommon presentations of CAA include the following:

  • CAA can be associated with ischemic strokes; in some of these patients, a coexistent vasculitis can be found; the causal relationship with CAA is unclear

  • CAA is found in patients with autosomal dominant dementia, spasticity, and ataxia without ICH

  • CAA is reported in patients with vascular malformations, postirradiation necrosis, spongiform encephalopathies, and dementia pugilistica

  • CAA can present as a mass lesion, such as an amyloidoma with accumulation of amyloid in the brain parenchyma, or as edema and gliosis secondary to the vascular lesion

  • CAA can manifest as a reversible leukoencephalopathy, with rapid progression of symptoms and imaging abnormalities, followed by dramatic improvement [7, 8]


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