Presentation
Woke with right facial droop and slurred speech. No haedache or reduced consciousness. Previous history of hypertension.
Patient Data
Small acute hematoma in the left frontal lobe subcortical white matter. There is no subarachnoid, subdural or ventricular hemorrhage. The hematoma has a smooth contour with no finger-like porjections. There is no significant mass effect.
Moderate periventricular low attenuation in keeping with small vessel disease. Mild atrophy.
Small area of signal drop out on the blood sensitive sequences consistent with the left frontal hematoma. There are multiple cerebral lobar microbleeds but no superficial siderosis . No other macrohaemorrhages or deep microbleeds.
Moderate periventricular white matter hyperintensities, marked basal ganglia enlarged perivascular spaces and moderate generalized cerebral volume loss.
Case Discussion
Left frontal lobar hemorrhage without extension into the subarachnoid space or finger-like projections. Background changes of lobar microbleeds, small vessel disease (enlarged perivascular spaces and white matter hyperintensities) and moderate atrophy.
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 uses 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 initial CT shows no subarachnoid hemorrhage or finger-like projections from the hematoma. The patient possessed at least one APOE e4 allele. Therefore they are intermediate 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 a single lobar macrohaemorrhage and multiple lobar microbleeds and so is probable CAA on the modified Boston criteria.
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PATHOLOGY: Post mortem three years later showed a large acute right sided hemorrhage and old left frontal hematoma. There is extensive small vessel disease throughout the white matter. Immunohistochemistry shows widespread amyloid angiopathy in the meningeal and parenchymal vessels.
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Cases such as this show that lobar hemorrhages often occur in the context of severe mixed vascular pathology (both cerebral amyloid angiopathy and white matter small vessel disease). Determining the precise cause can be difficult, and the hemorrhage may be related to multiple different pathologies, even those with MRI biomarkers of CAA.
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