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Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Mark Rodrigues
Diagnosis certain

Presentation

Found collapsed, unresponsive

Patient Data

Age: 80 years
Gender: Male

Large right frontal lobar hemorrhage involving cortex, subcortical white matter and periventricular white matter. There is subarachnoid and intraventricular 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 suprasellar cistern.

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

Case Discussion

Large right frontal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid and intraventricular 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 also 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.

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PATHOLOGY: Postmortem performed 14 months after the ICH showed an extensive right cerebral hematoma. Immunohistochemistry showed extensive parenchymal and widespread vascular depositions. Severe small vessel disease throughout the white matter with several lacunar infarcts.

 

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.  The overall appearances are more consistent with cerebral amyloid angiopathy-associated hemorrhage.

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