Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Found collapsed with GCS 3

Patient Data

Age: 80 years
Gender: Female

Large left frontal lobar hemorrhage involving cortex, subcortical white matter and periventricular white matter. There is overlying and distant subarachnoid hemorrhage, plus intraventricular hemorrhage. The hematoma has lobulations with finger-like projections evident (best seen on the sagittal plane - see stack key image).

Significant mass effect from the hematoma and perihaematomal edema resulting in midline shift and obstructive hydrocephalus of the lateral ventricles.

Mild periventricular low attenuation in keeping with small vessel change +/- transependymal CSF spread. Moderate cortical atrophy evident near vertex.

Case Discussion

Large left frontal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid and intraventircular 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 also 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.

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PATHOLOGY: Postmortem performed one day after the ICH showed a large left frontal hematoma extending to the caudate nucleus with extensive subarachnoid hemorrhage. Immunohistochemistry showed extensive leptomeningeal and parenchymal vessels plus amyloid plaques depositions (Braak and Braak stage 6). Widespread small vessel disease throughout the white matter with lipohyalinosis.

 

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.

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Case information

rID: 58752
Published: 5th Mar 2018
Last edited: 5th Mar 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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