Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Sudden onset left arm and leg weakness

Patient Data

Age: 85 years
Gender: Female

Large right frontoparietal lobar hemorrhage involving cortex and subcortical white matter. There is overlying and distant subarachnoid 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.

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

Case Discussion

Large right frontoparietal lobar hemorrhage with the involvement of the cortex and extension into the subarachnoid 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 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 three days after the ICH showed a large right frontoparietal hematoma extending to the subarachnoid space. Immunohistochemistry showed extensive leptomeningeal and parenchymal vessels plus amyloid plaques depositions (Braak and Braak stage 2). 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, although the location and pathological appearances are more in keeping with cerebral amyloid angiopathy.

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Case information

rID: 58751
Published: 5th Mar 2018
Last edited: 5th Mar 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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