by Kurt Jellinger

General outline

A rare form of cerebrovascular dementia caused by amyloid deposits in small-vessel walls which give rise to hemorrhages.

Synonyms

Congophilic angiopathy

Symptoms and course

A combination of neurological and psychopathological symptoms. Stepwise progressive, hemorrhages cause neurological symptoms accompanied by progressive dementia.

Causes and risk factors

Several mutations on the APP gene on Chromosome 21 have been identified in Dutch families.

Frequency

CAA at a subclinical level can occur in up to 98% of AD and 85% of aged brains.

Diagnostic procedures

Imaging (CCT and MRI) demonstrates the multiple hemorrhages. Genetic testing is used as ApoE [epsilon] 4 is a risk factor.

Care and treatment

Anticoagulants and thrombolysis; rehabilitation after stroke.

Available services

The Cerebral Amyloid Angiopathy (CAA) Resource This website is intended as a resource for both patients and families suffering from CAA and the investigators and clinicians who work in this field.

http://angiopathy.org

References

• Greenberg: Neurology 51 (1998) 690-94.