Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage
Presentation
Sudden onset headache, nausea and vomiting. Subsequent left arm and leg weakness
Patient Data
Large right lobar hemorrhage which is centered in the occipital lobe, but also involves the temporal and parietal lobes. It involves cortex, subcortical white matter and periventricular white matter. There is subarachnoid but no intraventricular or subdural hemorrhage. The hematoma has multiple finger-like projections (see stack key images).
Significant mass effect from the hematoma and perihaematomal edema resulting in midline shift, obstructive hydrocephalus of the lateral ventricles and partial effacement of the ipsilateral cortical sulci.
Mild periventricular low attenuation may represent small vessel change or transependymal CSF spread. Mild atrophy.
Case Discussion
Large right occipito-parieto-temporal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid. 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 did not possess 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 5 days after the ICH showed a large right occipital hematoma with extension into the subarachnoid and subdural spaces. Immunohistochemistry showed extensive parenchymal and widespread vascular depositions. Mild small vessel disease throughout the white matter with enlarged perivascular spaces.
The appearances are in keeping with a cerebral amyloid angiopathy-associated hemorrhage
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