Fact-sheet: Cerebral amyloid angiopathy


Updated on 04/30/2026 at 9:33 AM

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Definition

Deposits of A-beta amyloid peptides (40 and 42) in the walls of small and medium-sized arteries, weakening them, or more rarely in capillaries or veins, causing vascular dysfunction predominantly in the occipital regions where the vasculopathy is most pronounced.

Sporadic disease, more common in the elderly (>70 years), in patients with Alzheimer's disease, and in Trisomy 21.

Clinical features

Asymptomatic (50% of cases).
Recurrent cerebral hemorrhages with spontaneous lobar hematomas.
Amyloid spells: transient regressive neurological phenomena (TFNE) lasting a few minutes (positive or negative).
Progressive cognitive decline and stepwise progressive dementia.
Ischemic events.
Inflammatory form:

  • Rare manifestation.
  • Reversible encephalopathy, mean age at onset 63 years.
  • Acute or subacute onset headache, cognitive or behavioral disturbances, seizures, or focal neurological deficit.

CT

Edinburgh criteria on non-contrast CT (PPV 92%):

  • Lobar hematoma with finger-like extension
  • Subarachnoid hemorrhage associated with the hematoma
  • APOE4 genotype

Always perform a contrast-enhanced acquisition to search for another etiology.

MRI

Most appropriate complementary examination.
Modified Boston criteria (2022), current reference standard for diagnosing cerebral amyloid angiopathy in vivo:
PROBABLE CEREBRAL AMYLOID ANGIOPATHY:

  • Age >= 50 years
  • and transient focal neurological episodes (TFNE) or cognitive deficit or dementia
  • and single intraparenchymal hemorrhage
  • and at least two lobar hemorrhagic lesions on T2* among: Lobar hematoma
  • Microbleed
  • Superficial cortical siderosis
  • Subarachnoid hemorrhage

OR

  • Age >= 50 years
  • and a lobar hematoma with white matter lesions (severe dilation of the Virchow-Robin spaces of the centrum semiovale or multiple hyperintensities)
  • and absence of deep hemorrhagic lesion (deep hematoma - microbleed) on T2*
  • and absence of another cause of cerebral hemorrhage (see differential diagnoses)
  • Nota Bene: Cerebellar hemorrhagic lesions count neither as lobar lesions nor as deep lesions

POSSIBLE CEREBRAL AMYLOID ANGIOPATHY:

  • Age >= 50 years
  • and transient focal neurological episodes (TFNE) or cognitive deficit or dementia
  • and ONE lobar hemorrhagic lesion on T2* among: Lobar hematoma
  • Microbleed
  • Superficial cortical siderosis
  • Subarachnoid hemorrhage
  • and absence of another cause of cerebral hemorrhage

OR

  • Age >= 50 years
  • and white matter lesions (severe dilation of the Virchow-Robin spaces of the centrum semiovale or multiple hyperintensities)
  • and absence of deep hemorrhagic lesion (deep hematoma - microbleed) on T2*
  • and absence of another cause of cerebral hemorrhage (see differential diagnoses)
  • Nota Bene: Cerebellar hemorrhagic lesions count neither as lobar lesions nor as deep lesions

In practice, numerous T2* hypointense microbleeds of lobar distribution, preferentially occipital, spontaneous lobar hematomas, hemosiderin deposits at the cortical surface as a T2 gradient-echo hypointense rim, and leukoencephalopathy of predominantly posterior distribution, microinfarcts, dilation of the Virchow-Robin perivascular spaces.

INFLAMMATORY FORM
Diagnostic criteria allowing brain biopsy to be avoided (Auriel et al., 2016):
PROBABLE INFLAMMATORY CEREBRAL AMYLOID ANGIOPATHY
1. Age >40 years
2. Presence of at least one of the following clinical signs: headache, altered consciousness, behavioral change, focal deficit, seizure; not attributable to acute cerebral hemorrhage
3. Brain MRI shows unifocal or multifocal white matter lesions (cortico-subcortical or deep) that are asymmetric and extend to the immediately subcortical white matter (U-fibers); the asymmetry is not due to prior cerebral hemorrhage
4. Presence of at least one of the following cortico-subcortical hemorrhagic lesions: lobar hematoma, lobar microbleed, or superficial cortical siderosis
5. Absence of neoplastic, infectious, or any other cause
POSSIBLE INFLAMMATORY CEREBRAL AMYLOID ANGIOPATHY
Identical except for criterion 3 (absence of asymmetry)
3. MRI shows white matter lesions extending to the immediately subcortical white matter (U-fibers).
Possible contrast enhancement with perilesional edema and mass effect in pseudotumoral forms.
Possible associated hydrocephalus due to secondary meningeal dysfunction from vascular involvement.
Regression of inflammatory cerebral amyloid angiopathy lesions with treatment.

Management

Blood pressure control
Inflammatory form: corticosteroid therapy for 3 months

Differential diagnosis

  • Other causes of cerebral hemorrhage to exclude: Trauma
  • Ischemic stroke with hemorrhagic transformation and hypertensive hemorrhagic stroke
  • Cerebral arteriovenous malformation
  • Hemorrhagic tumor
  • Central nervous system vasculitis
  • Causes of cortical siderosis
  • Causes of subarachnoid hemorrhage, including intracranial aneurysm
  • Multiple cavernous angiomas
  • Diffuse axonal injury
  • CADASIL - Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leucoencephalopathy
  • Posterior reversible encephalopathy syndrome (PRES)
  • Beware of calcifications, which also appear hypointense on the T2* sequence

 

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