Cerebral amyloid angiopathy-associated lobar intracerebral haemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

3 days worsening headache. Sudden onset, associated vomiting and confusion.

Patient Data

Age: 70 years
Gender: Female

Acute right temporal lobe haematoma involving cortex and subcortical white matter. There is extension into the overlying subarachnoid space but no sub/extradural or intraventricular blood. Ovoid in shape with no finger-like projections. Mild localised mass effect.

Severe periventricular and deep white matter low attenuation in keeping with small vessel change.

Signal drop out on blood sensitive sequences at the site of right temporal ICH. Interval right frontal ICH has occurred. There is superficial siderosis over the right parietal and frontal lobes. No microbleeds.

Moderate to severe periventricular and deep white matter hyperintensities. Basal ganglia, midbrain, hippocampal and centrum semiovale enlarged perivascular spaces.

CT

CT performed 2.5 years after initial presentation

Large left temporal haematoma with extension into the subarachnoid and subdural spaces. The haematoma is lobulated. There is localised mass effect and some midline shift. No intraventricular blood.

Gliosis in the right frontal and temporal lobes at the sites of previous ICHs. Severe periventricular and deep white matter low attenuation in keeping with small vessel change.

Case Discussion

Left temporal lobar haemorrhage with involvement of the cortex, extension into the subarachnoid space but without finger-like projections on the initial CT. Background changes of cortical superficial siderosis and small vessel disease (enlarged perivascular spaces and white matter hyperintensities). Recurrent right frontal ICH, and left temporal ICH with subarachnoid haemorrhage and 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 initial CT shows subarachnoid haemorrhage but no finger-like projections from the haematoma.  The patient 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.

This patient has multiple lobar macrohaemorrhages and cortical superficial siderosis and so is probable CAA on the modified Boston criteria.

---

PATHOLOGY: Post mortem showed left temporal haematoma, old right frontal and temporal haematomas. Extensive cerebral amyloid angiopathy throughout the cerebrum and cerebellum, severe small vessel disease and Alzheimer-type pathology.

PlayAdd to Share

Case information

rID: 58378
Published: 14th Feb 2018
Last edited: 22nd Feb 2018
Inclusion in quiz mode: Included
Institution: University of Edinburgh

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.