Amyloid angiopathy with inflammation

Discussion:

This case highlights several imaging features of cerebral amyloid angiopathy, namely micro-hemorrhages, superficial siderosis, infarcts and amyloid related inflammation.

Imaging features of cerebral amyloid angiopathy or 'amyloid related imaging abnormalities' (ARIA) have been loosely divided into 2 groups, ARIA-H (hemorrhage) and ARIA-E (edema/edema).

Amyloid related hemorrhage (ARIA-H) has several manifestations:

  • micro-hemorrhages, typically sub-cortical in distribution, correlating with disease progression and cognitive impairment
  • lobar hematoma, in the same distribution as micro-hemorrhages
  • sub-arachnoid hemorrhage, typically at the vertex and often associated clinically with seizures and/or TIA like symptoms 
  • superfical siderosis, the result of sub-arachnoid hemorrhage, frequently at cerebral vertex, rarely in the posterior fossa

Amyloid related inflammation with edema (ARIA-E):

  • is a form of vasculitis
  • may be associated with headache, cognitive decline and seizures
  • manifests on imaging as confluent white matter T2 signal change, possibly involving cortex and with mass effect
  • is potentially treatable - may respond to immunosuppression 

Infarcts in patients with cerebral amyloid angiopathy are increasingly recognized as a manifestation of the disease. They probably reflect occlusive arteriopathy, and may be clinically silent. 

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