Alzheimer disease and cerebral amyloid angiopathy

Case contributed by Assoc Prof Frank Gaillard

Presentation

Progressive cognitive decline particularly short-term memory.

Patient Data

Age: 75 years

Bilateral biopsy tracks are again noted in the frontal lobes.

Coronal volumetric scans show atrophy of the hippocampi out of keeping with the changes in the rest of the brain. Multiple small foci of signal dropout on the susceptibility weighted imaging are in keeping with the suspected diagnosis of amyloid angiography. No evidence of superficial siderosis. 

Severe abnormality is present in the periventricular and subcortical white matter. No evidence of restricted diffusion or contrast enhancement in these regions. MRA (not shown) remains normal. MR perfusion (not shown) suggests regions of decreased perfusion in the temporoparietal lobes

Conclusion: Overall, features suggest a diagnosis of Alzheimer's disease with associated amyloid angiography

Case Discussion

There was a concern of a vasculitis in this patient and as a result, they underwent two brain biopsies. 

Histology

Left frontal lobe biopsy. Review of the sections and immunostaining shows strong A-beta protein deposition in the walls of cortical blood vessels. In addition, there are numerous mature plaques with strong A-beta staining. The features are of cerebral amyloid angiopathy and probable Alzheimer's disease. Immunostaining for prion protein is NEGATIVE.

The areas of high T2 signal that resolved (not shown) likely represented cerebral amyloid-related inflammation. 

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