Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Severe headache. Then developed left sided weakness with confusion

Patient Data

Age: 90 years
Gender: Female

Large right temporal lobar hemorrhage involving cortex, subcortical white matter and periventricular white matter. There is overlying and distant subarachnoid and subdural, plus intraventricular hemorrhage. The hematoma has lobulations with finger-like projections evident (see stack key images).

Mass effect from the hematoma and perihaematomal edema resulting in minor midline shift.

Mild periventricular low attenuation in keeping with small vessel change. Moderate cortical atrophy evident near vertex.

Case Discussion

Large right temporal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid, subdural and intraventricular spaces. The hematoma contains multiple finger-like projections.

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 Edinburgh CT and genetic diagnostic criteria for lobar intracerebral hemorrhage associated with cerebral amyloid angiopathy use CT features (presence of subarachnoid hemorrhage, finger-like projections arising from the ICH) and APOE e4 genotype (if available) to classify a patient as high, intermediate or low risk of CAA-associated ICH. The CT shows subarachnoid hemorrhage and finger-like projections from the hematoma. The patient did not have an 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.

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PATHOLOGY: Postmortem performed 6 days after the ICH showed a large right temporal, parietal and occipital hematoma extending through the white matter and to the cortex.  There is focal subarachnoid hemorrhage. Immunohistochemistry showed extensive amyloid in the leptomeningeal and parenchymal vessels plus amyloid plaques depositions (Braak and Braak stage 3). Moderate small vessel disease throughout the white matter with lipohyalinosis.

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This case highlights that the small vessel diseases underlying lobar ICH is often mixed. The hemorrhage may have been related to arteriolosclerosis or cerebral amyloid angiopathy. The findings are most in keeping with a cerebral amyloid angiopathy associated hemorrhage.

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Case information

rID: 58758
Published: 5th Mar 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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