Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Found confused, agitated and disorientated by daughter

Patient Data

Age: 80 years
Gender: Female

Small left temporal lobar hemorrhage involving cortex and subcortical white matter. There is overlying subarachnoid hemorrhage. The hematoma has lobulations with finger-like projections evident (best seen on the coronal plane - see stack key image).

No significant mass effect from the hematoma or perihaematomal edema.

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

CT

CT (3 months after initial presentation) due to agitation, confusion and right sided weakness

Large acute hematoma in the left parietal, occipital and temporal lobes. Associated subarachnoid hemorrhage and lobulated hematoma contour (finger-like projections).

Mass effect from the heamatoma and perihaematomal edema.

Case Discussion

Small left temporal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid space, and finger-like projections.  Recurrent left parieto-occipito-temporal hematoma with subarachnoid hemorrhage and 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 three months after the initial ICH showed a large left sided cerebral hematoma extending to the subarachnoid space. Immunohistochemistry showed severe leptomeningeal and parenchymal vessels amyloid deposition plus amyloid plaques depositions (Braak and Braak stage 2). Mild 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 appearances are in keeping with a cerebral amyloid angiopathy associated hemorrhage.

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Case information

rID: 58757
Published: 5th Mar 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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