Amyloid angiopathy with inflammation

Case contributed by Prof. Stephen Stuckey

Presentation

Expressive dysphasia and visual disturbance.

Patient Data

Age: 80 years
Gender: Male
Modality: MRI

Confluent areas of sub-cortical and deep white matter high T2 signal within the left frontal, parietal and temporal lobes. Mild positive mass effect in the left frontal lobe. Punctate foci of restricted diffusion in left frontal and parietal lobes. Superficial siderosis of both cerebral hemispheres and multiple intraparenchymal sub-cortical microhaemorrhages.

Case Discussion

This case highlights several imaging features of cerebral amyloid angiopathy, namely micro-haemorrhages, 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 (haemorrhage) and ARIA-E (edema/oedema).

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

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

Amyloid related inflammation with oedema (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 recognised as a manifestation of the disease. They probably reflect occlusive arteriopathy, and may be clinically silent. 

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Case Information

rID: 30360
Case created: 5th Aug 2014
Last edited: 22nd Feb 2017
Inclusion in quiz mode: Included

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