Cerebral amyloid angiopathy-related inflammation

Case contributed by Y. Amy Chen
Diagnosis almost certain

Presentation

Several months history of headaches and progressive cognitive decline. No fever.

Patient Data

Age: 65 years
Gender: Male

There are diffuse FLAIR hyperintense white matter signal abnormalities in bilateral temporal lobes and left frontal lobe. No restricted diffusion. However, these regions demonstrate increase diffusivity on ADC which corresponds to vasogenic edema. SWI images demonstrate multiple punctate foci of susceptibility in the cortex and juxtacortex due to microhemorrhages.

Case Discussion

This case is most consistent with cerebral amyloid angiopathy-related inflammation. Patients are usually elderly and tend to present with subacute fatigue, confusion, and cognitive decline. Blood and CSF evaluation including infectious and inflammatory markers are often normal. Development of inflammatory changes in patients with CAA is more frequent in those with the APOE ε4 genotype. Treatment is with high dose steroids and patients usually improve.

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