Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Difficulty with memory, problems with reversing car and sequencing tasks and confusion. Sudden onset expressive dysphasia and right sided weakness

Patient Data

Age: 60 years
Gender: Male

Acute left frontal hematoma involving the cortex and subcortical white matter. There is localized and subarachnoid hemorrhage.  No subdural, extradural or intraventricular component. The haemtoma has a regular contour with no finger-like projections.

Mild mass effect.

Further subacute left frontal hemorrhage. Bifrontal gliotic regions ion keeping with previous infarcts (hemorrhage or ischemic). Minor periventricular low attenuation in keeping with small vessel disease.  Mild generalized cerebral volume loss.

Large area of signal drop out on the blood sensitive sequences consistent with the left frontal hematoma. The other bifrontal abnormalities have signal drop out in keeping with further hematomata. There is superficial siderosis overlying the both cerebral hemispheres and multiple cerebral microbleeds.  No other macrohaemorrhages or deep microbleeds.

Mild periventricular white matter hyperintensities, basal ganglia and centrum semiovale enlarged perivascular spaces and mild generalized cerebral volume loss.

New bilateral frontal/parietal acute hematomata with acute cerebral convexity subarachnoid hemorrhage.

Previously demonstrated bilateral cerebral hematomata now have a subacute appearance. Further acute bilateral lobar hemorrhages with acute subarachnoid hemorrhage

Case Discussion

Left frontal lobar hemorrhage with involvement of the cortex and extension into the subarachnoid space. Background changes of multiple old lobar hemorrhages and cortical superficial siderosis. Mild small vessel disease (enlarged perivascular spaces and white matter hyperintensities) and atrophy. Multiple recurrent lobar hemorrhages with subarachnoid hemorrhage.

Lobar intracerebral hemorrhage is frequently attributed to small vessel diseases (cerebral amyloid angiopathy or arteriolosclerosis).  Differentiating lobar hemorrhage due to cerebral amyloid angiopathy and arteriolosclerosis is important due to differences in recurrent ICH and post-stroke dementia risk (higher with CAA-associated ICH).

The initial CT shows subarachnoid hemorrhage but no finger-like projections from the hematoma. The patient possessed at least one APOE e4 allele. Therefore they are high risk for CAA-associated ICH on the Edinburgh CT and genetic diagnostic criteria for lobar intracerebral hemorrhage associated with cerebral amyloid angiopathy.

This patient has a multiple lobar macrohaemorrhages and cortical superficial siderosis and so is probable CAA on the modified Boston criteria.

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PATHOLOGY: Post mortem showed multiple intracerebral hemorrhages of different ages with subarachnoid hemorrhage.  Multiple microbleeds are present. The vessels in the leptomeninges and cortex show extensive cerebral amyloid angiopathy along with capillary amyloid deposition. Parenchymal amyloid deposition is present but not as prominent as would be expected for the degree of vascular amyloid.

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Case information

rID: 58529
Published: 22nd Feb 2018
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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