Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage
Presentation
3 days worsening headache. Sudden onset, associated vomiting and confusion.
Patient Data
Acute right temporal lobe hematoma involving cortex and subcortical white matter. There is extension into the overlying subarachnoid space but no sub/extradural or intraventricular blood. Ovoid in shape with no finger-like projections. Mild localized mass effect.
Severe periventricular and deep white matter low attenuation in keeping with small vessel change.
Signal drop out on blood sensitive sequences at the site of right temporal ICH. Interval right frontal ICH has occurred. There is superficial siderosis over the right parietal and frontal lobes. No microbleeds.
Moderate to severe periventricular and deep white matter hyperintensities. Basal ganglia, midbrain, hippocampal and centrum semiovale enlarged perivascular spaces.
CT 2.5 yr after initial...
CT 2.5 yr after initial presentation
Large left temporal hematoma with extension into the subarachnoid and subdural spaces. The hematoma is lobulated. There is localized mass effect and some midline shift. No intraventricular blood.
Gliosis in the right frontal and temporal lobes at the sites of previous ICHs. Severe periventricular and deep white matter low attenuation in keeping with small vessel change.
Case Discussion
Left temporal lobar hemorrhage with involvement of the cortex, extension into the subarachnoid space but without finger-like projections on the initial CT. Background changes of cortical superficial siderosis and small vessel disease (enlarged perivascular spaces and white matter hyperintensities). Recurrent right frontal ICH, and left temporal ICH 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 initial CT shows subarachnoid hemorrhage but no finger-like projections from the hematoma. The patient 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.
This patient has multiple lobar macrohaemorrhages and cortical superficial siderosis and so is probable CAA on the modified Boston criteria.
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PATHOLOGY: Post mortem showed left temporal hematoma, old right frontal and temporal hematomas. Extensive cerebral amyloid angiopathy throughout the cerebrum and cerebellum, severe small vessel disease and Alzheimer-type pathology.
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