Lobar intracerebral haemorrhage
Woke with right facial droop and slurred speech. No haedache or reduced consciousness. Previous history of hypertension.
Loading Stack -
0 images remaining
Small acute haematoma in the left frontal lobe subcortical white matter. There is no subarachnoid, subdural or ventricular haemorrhage. The haematoma has a smooth contour with no finger-like porjections. There is no significant mass effect.
Moderate periventricular low attenuation in keeping with small vessel disease. Mild atrophy.
Loading Stack -
0 images remaining
Small area of signal drop out on the blood sensitive sequences consistent with the left frontal haematoma. There are multiple cerebral lobar microbleeds but no superficial siderosis . No other macrohaemorrhages or deep microbleeds.
Moderate periventricular white matter hyperintensities, marked basal ganglia enlarged perivascular spaces and moderate generalised cerebral volume loss.
Left frontal lobar haemorrhage without extension into the subarachnoid space or finger-like projections. Background changes of lobar microbleeds, small vessel disease (enlarged perivascular spaces and white matter hyperintensities) and moderate atrophy.
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 uses 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 no subarachnoid haemorrhage or finger-like projections from the haematoma. The patient possessed at least one APOE e4 allele. Therefore they are intermediate 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 a single lobar macrohaemorrhage and multiple lobar microbleeds and so is probable CAA on the modified Boston criteria.
PATHOLOGY: Post mortem three years later showed a large acute right sided haemorrhage and old left frontal haematoma. There is extensive small vessel disease throughout the white matter. Immunohistochemistry shows widespread amyloid angiopathy in the meningeal and parenchymal vessels.
Cases such as this show that lobar haemorrhages often occur in the context of severe mixed vascular pathology (both cerebral amyloid angiopathy and white matter small vessel disease). Determining the precise cause can be difficult, and the haemorrhage may be related to multiple different pathologies, even those with MRI biomarkers of CAA.
- Rodrigues MA, Samarasekera N, Lerpiniere C, Humphreys C, McCarron MO, White PM, Nicoll JAR, Sudlow CLM, Cordonnier C, Wardlaw JM, Smith C, Al-Shahi Salman R. The Edinburgh CT and genetic diagnostic criteria for lobar intracerebral haemorrhage associated with cerebral amyloid angiopathy: model development and diagnostic test accuracy study. (2018) The Lancet. Neurology. 17 (3): 232-240. doi:10.1016/S1474-4422(18)30006-1 - Pubmed
- Samarasekera N, Smith C, Al-Shahi Salman R. The association between cerebral amyloid angiopathy and intracerebral haemorrhage: systematic review and meta-analysis. (2012) Journal of neurology, neurosurgery, and psychiatry. 83 (3): 275-81. doi:10.1136/jnnp-2011-300371 - Pubmed
- Pantoni L. Cerebral small vessel disease: from pathogenesis and clinical characteristics to therapeutic challenges. (2010) The Lancet. Neurology. 9 (7): 689-701. doi:10.1016/S1474-4422(10)70104-6 - Pubmed
- Linn J, Halpin A, Demaerel P, Ruhland J, Giese AD, Dichgans M, van Buchem MA, Bruckmann H, Greenberg SM. Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. (2010) Neurology. 74 (17): 1346-50. doi:10.1212/WNL.0b013e3181dad605 - Pubmed