Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Mark Rodrigues
Diagnosis certain

Presentation

2 week history of headaches, new onset dysphasia, reduced GCS

Patient Data

Age: 75 years
Gender: Male

Moderate left temporal lobar hemorrhage involving cortex and subcortical white matter. There is diffuse subarachnoid hemorrhage. The hematoma has an irregular outline with finger-like projections visible (best seen on the sagittal plane - see stack key images).

Focus of less marked hyperattenuation in the right temporal lobes, which is in keeping with a subacute hemorrhage.

No significant mass effect.

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

Reduction in GCS during rehabilitation

New acute interecerebral hemorrhage in the left parietal lobe with associated subarachnoid hemorrhage. Less acute right parietal hematoma. Gliotic regions in the temporal lobes related to the previous hemorrhages.

Background small vessel change and moderate atrophy as before.

Case Discussion

Multiple lobar hemorrhages of differing ages with involvement of the cortex, extension into the subarachnoid spaces. The left temporal 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 acute 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.

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PATHOLOGY: Postmortem performed 2 months after the initial presentation showed several lobar hematomas of differing ages. Widespread subarachnoid hemorrhage is present. an extensive right cerebral hematoma. Immunohistochemistry showed extensive parenchymal and widespread vascular depositions. small vessel disease throughout the white matter in the form of lipohyalinosis and arteriolosclerosis, plus 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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