Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Sudden onset headache with left sided weakness. Vomiting

Patient Data

Age: 75 years
Gender: Female

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.

Mass effect from the hematoma and perihaematomal edema with generalized effacement of the CSF spaces but no midline shift or hydrocephalus.

Moderate periventricular low attenuation in keeping with small vessel change. Mild atrophy.

New left frontal intracerebral hematoma.  Subdural and subarachnoid hemorrhage present. Small volume ventricular hemorrhage. 

Right frontal gliosis with persistant sulcal effacement relating to the previous hemorrhage.

Interval left frontal croniotomy with linear low attenuation extending to the left lateral ventricle frontal horn in keeping with previous ventricular access device placement.

The CSF spaces remained effaced.

Case Discussion

Right frontal lobar hemorrhage with the involvement of the cortex, extension into the subarachnoid and intraventricular spaces. The hematoma contains multiple finger-like projections. Recurrent left frontal hematoma with subarachnoid and subdural hemorrhage.

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 3 months after the ICH showed bifrontal cerebral hematomas with subarachnoid hemorrhage. Immunohistochemistry showed extensive parenchymal (Braak and Braak stage 3-4) and widespread vascular depositions. Moderate 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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Case information

rID: 58566
Published: 23rd Feb 2018
Last edited: 24th Feb 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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