Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Mark Rodrigues
Diagnosis certain

Presentation

Sudden onset left sided weakness and neglect. No past medical history

Patient Data

Age: 85 years
Gender: Male

Large right parietal lobar hemorrhage involving cortex, subcortical white matter and periventricular white matter. There is subarachnoid, subdural and intraventricular hemorrhage. The hematoma has a lobulated contour without finger-like projections.

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

Moderate periventricular low attenuation in keeping with small vessel change. Moderate cortical atrophy evident near vertex. Gliosis inferiorly in the right frontal lobe may represent an old infarct or traumatic brain injury.

Case Discussion

Large right parietal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid, subdural and intraventricular spaces. The hematoma contains lobulations without distinct 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 but no finger-like projections from the hematoma. The patient possessed 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 3 weeks after the ICH showed a large right posterior frontal and parietal hematoma involving the cortex and white matter.  There is focal subarachnoid hemorrhage. Immunohistochemistry showed widespread amyloid in the leptomeningeal and parenchymal vessels plus amyloid plaques depositions. There is extensive small vessel disease throughout the white and deep grey 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.

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