Cerebral amyloid angiopathy-associated lobar intracerebral haemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Found collapsed and unresponsive with right sided weakness

Patient Data

Age: 70 years
Gender: Female

Large left frontal lobar haemorrhage involving cortex, subcortical white matter and periventricular white matter. There is overlying subarachnoid haemorrhage and a left subdural haemorrhage. The haematoma has lobulations with finger-like projections evident (best seen on the sagittal plane - see stack key image).

Significant mass effect from the haematoma and perihaematomal oedema resulting in midline shift and obstructive hydrocephalus of the lateral ventricles.

Severe periventricular low attenuation in keeping with small vessel change +/- transependymal CSF spread. Mild atrophy. Chronic right occipital infarct

Case Discussion

Large left frontal lobar haemorrhage with the involvement of the cortex, extension into the subarachnoid and subdural 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 CT shows subarachnoid haemorrhage and finger-like projections from the haematoma. 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 haemorrhage associated with cerebral amyloid angiopathy.

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PATHOLOGY: Postmortem performed one week after the ICH showed a large left frontal haematoma with extensive subarachnoid haemorrhage. Immunohistochemistry showed extensive leptomeningeal and parenchymal vessels plus amyloid plaquesdepositions. Widespread small vessel disease throughout the white matter with lipohyalinosis.

 

This case highlights that the small vessel diseases underlying lobar ICH is often mixed. The haemorrhage may have been related to arteriolosclerosis or cerebral amyloid angiopathy.  The overall appearances are more consistent with cerebral amyloid angiopathy-associated haemorrhage.

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Case information

rID: 58561
Published: 23rd Feb 2018
Last edited: 24th Feb 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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