Cerebral amyloid angiopathy-associated lobar intracerebral hemorrhage

Case contributed by Dr Mark Rodrigues

Presentation

Woke up with right arm weakness

Patient Data

Age: 80 years
Gender: Male

Acute left frontal hematoma involving the cortex and subcortical white matter. There is localized and distant (anterior interhemispheric fissure) subarachnoid hemorrhage.  No subdural, extradural or intraventricular component. The haemtoma has a lobulated contour but no finger-like projection.

Mild mass effect.

Mild periventricular low attenuation in keeping with small vessel disease.  Moderate generalized cerebral volume loss.

Large area of signal drop out on the blood sensitive sequences consistent with the left frontal hematoma. There is superficial siderosis overlying the left cerebral hemisphere and multiple cerebral microbleeds.  No other macrohaemorrhages or deep microbleeds.

Mild periventricular white matter hyperintensities, basal ganglia enlarged perivascular spaces and moderate generalized cerebral volume loss.

CT

CT performed 2.5 years after initial presentation due to left arm weakness

Acute hemorrhage in the right frontal and parietal lobes involving cortex and subcortical white matter, with overlying subarachnoid hemorrhage. No significant mass effect.

Gliosis in the left frontal lobe at the site of previous ICH. Further gliosis in the right frontal lobe may represent a previous ICH or ischemic infarct.

Marked progression in the periventricular white matter lucencies consistent with small vessel change and generalized cerebral volume loss.

Case Discussion

Left frontal lobar hemorrhage with involvement of the cortex and extension into the subarachnoid space. Background changes of cortical superficial siderosis, lobar microbleedssmall vessel disease (enlarged perivascular spaces and white matter hyperintensities) and moderate atrophy. Recurrent right frontoparietal ICH with subarachnoid 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 initial CT shows subarachnoid hemorrhage but no finger-like projections from the hematoma. 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 hemorrhage associated with cerebral amyloid angiopathy.

This patient has a single lobar macrohaemorrhage, cortical superficial siderosis and multiple lobar microbleeds and so is probable CAA on the modified Boston criteria.

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PATHOLOGY: Post mortem showed right and left fronto-parietal hematomata.  Acute subarachnoid hemorrhage and superficial siderosis. The vessels in the leptomeninges and cortex show extensive cerebral amyloid angiopathy along with Alzheimer's type pathology (Braak and Braak stage 3). Only mild small vessel disease is present in the white matter.

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Case information

rID: 58376
Published: 14th Feb 2018
Last edited: 24th Jan 2019
Inclusion in quiz mode: Included
Institution: University of Edinburgh

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