Cerebral amyloid angiopathy

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

Previous occipital hemorrhage.

Patient Data

Age: 60
Gender: Female
mri

A new right sub dural hematoma is demonstrated, with acute on chronic features and blood products layering dependently. This measures approximately 10 mm in maximum diameter and results in mild mass effect on the fronto parietal parenchyma. No significant midline shift.

Previous left occipital craniotomy. Encephalomalacia, with hemosiderin staining within the left occipital lobe, with adjacent associated FLAIR signal in region of previous hematoma. Superficial siderosis as demonstrated on susceptibility imaging seen in the parieto-occipital regions and spires in the right frontal lobe, consistent with previous hemorrhage.

In addition to this, numerous bilateral punctate peripheral foci of susceptibility change are consistent with micro hemorrhages related to amyloid angiopathy.

 

 

Case Discussion

The patient had had a previous biopsy of the left occipital bleed (acutely)

Histology

MICROSCOPIC DESCRIPTION:

The section shows fragments of edematous cerebral cortex with attached blood clot. In the blood clot there are several arterial and venous channels. Within the cortex and leptomeninges there are prominent blood vessels with thickened hyalinised walls. These show strong immunostaining for A-beta protein. Many mature neuritic plaques are also noted in cerebral cortex. The features are of cerebral amyloid angiopathy with hemorrhage. No evidence of tumor is seen.

DIAGNOSIS:

A-beta cerebral amyloid angiopathy with hemorrhage 

COMMENT:

The number of neuritic plaques identified in cerebral cortex in the A-beta immunostained section is above what would be expected in an individual aged 57 years. This finding raises the possibility of early onset Alzheimer's disease. Formal cognitive testing is recommended. If this is abnormal, genetic testing for presinilin 1 and 2 mutations would be indicated.

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