Cerebral amyloid angiopathy-associated lobar intracerebral haemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Sudden onset right side weakness plus vomiting

Patient Data

Age: 85 years
Gender: Male

Large left frontal lobar haemorrhage involving cortex, subcortical white matter and periventricular white matter. There is subarachnoid and intraventricular haemorrhage. The haematoma has multiple finger-like projections.

Mass effect from the haematoma and perihaematomal oedema resulting in midline shift, obstructive hydrocephalus of the lateral ventricles and partial effacement of the suprasellar cistern.

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

Case Discussion

Large left frontal lobar haemorrhage with the involvement of the cortex, extension into the subarachnoid and intraventricular spaces. The haematoma contains multiple finger-like projections.

Lobar intracerebral haemorrhage is frequently attributed to small vessel diseases (cerebral amyloid angiopathy or arteriolosclerosis).  Differentiating lobar haemorrhage 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 haemorrhage associated with cerebral amyloid angiopathy use CT features (presence of subarachnoid haemorrhage, 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 haemorrhage and finger-like projections from the haematoma. The patient did not possess an APOE e4 allele. Therefore they are high risk for CAA-associated ICH on the Edinburgh CT and genetic diagnostic criteria for lobar intracerebral haemorrhage associated with cerebral amyloid angiopathy.

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PATHOLOGY: Postmortem performed 8 days after the ICH showed an extensive left cerebral haematoma with subarachnoid haemorrhage. Immunohistochemistry showed extensive parenchymal (Braak and Braak stage 5) and widespread vascular depositions (cerebral amyloid angiopathy). Extensive 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 haemorrhage may have been related to arteriolosclerosis/lipohyalinosis or cerebral amyloid angiopathy.

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Case information

rID: 58567
Published: 23rd Feb 2018
Last edited: 24th Feb 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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