Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Mark Rodrigues
Diagnosis certain

Presentation

Collapsed with reduced GCS 4/15.

Patient Data

Age: 80 years
Gender: Female

Large right lobar hemorrhage (frontal and parietal lobes) involving cortex, subcortical white matter and periventricular white matter. There is subarachnoid and intraventricular hemorrhage. The hematoma has multiple finger-like projections.

Significant mass effect resulting in midline shift and obstructive hydrocephalus of the lateral ventricles.

Mild periventricular low attenuation may represent small vessel change or transependymal CSF spread.

Case Discussion

Large right frontoparietal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid and subdural spaces. 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 initial CT shows subarachnoid hemorrhage and finger-like projections from the hematoma. The patient did not possess 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.

The large ICH volume, combined with reduced GCS on admission, the patient's age (80 years) and intraventricular extension were poor prognostic factors (ICH score 5).  The patient died the same day. 

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PATHOLOGY: Postmortem showed an extensive right cerebral hematoma with subarachnoid and ventricular extension. There was extensive cerebral amyloid angiopathy plus severe small vessel disease.

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