Cerebral amyloid angiopathy

Case contributed by RMH Neuropathology

Presentation

Progressive dementia and right sided weakness.

Patient Data

Age: 51
Gender: Male
MRI

Focus of cortical loss and subjacent subcortical T2 hyperintensity in the left superior and middle frontal gyrus. In the left centrum semiovale, corona radiata and mid body of corpus callosum deep to this region there are several small foci of diffusion restriction. These were present on the previous MRI from 3 October, 2013 (not shown). No associated contrast enhancement or foci of susceptibility artefact..

Scattered periventricular, deep and subcortical T2 hyperintense foci elsewhere in cerebral white matter are unchanged. These are in excess of expected lesion number for age. Punctate focus of susceptibility artefact in the left anterior temporal lobe and in the left parietal lobe, one in the right temporal lobe and several in the inferior frontal lobes are present, typical of microhemorrhages secondary to cerebral amyloid angiopathy. 

No pathological contrast enhancement. No mass lesion identified. Normal MRA (not shown). Foetal supply of the posterior cerebral arteries is incidentally noted.

Conclusion:

Cerebral amyloid angiopathy, with superimposed left frontal encephalomalacic insult. Presence of restricted diffusion is of uncertain significance. CAA related inflammation / vasculitis is a possibility.

Catheter angiography (not shown): There is no angiographic abnormality.In particular, there is no evidence of vasculitis.

MICROSCOPIC DESCRIPTION:

The sections contain cerebral cortex and white matter. A few of the leptomeningeal and cortical blood vessels contain amorphous eosinophilic material within the walls (Fig 1 - A&B). There is no evidence of thrombosis or vasculitis. No haemorrhage or infarcts are noted.

The Congo red stain (Fig 1 - C) is positive in the blood vessels for amyloid. It shows apple-green birefringence under polarised light (Fig 1 - D).

The B-amyloid immunostain (Fig 2 - A&B) is also positive in the vessels. It highlights several amyloid plaques in the cortex. These are not significantly increased in numbers. Assessment for dementia/Alzheimer's disease would require more dedicated biopsy samples.

No evidence of granulomatous angiitis to suggest CAA related inflammation. (Fig 2 - C) if from a different patient, highlighting the appearances of granulomatous angiitis. 

DIAGNOSIS:

Right frontal cortex biopsy: Amyloid angiopathy.

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Case information

rID: 28698
Case created: 8th Apr 2014
Last edited: 2nd Nov 2015
Inclusion in quiz mode: Included

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