Dutchtype CAA is a genetic disease. When one of your parents carries the gene, you have a 50% chance of also being a gene carier. Sporadic CAA is not hereditary and is a cause of dementia in about 1 in 4 elderly worldwide.
The disease CAA (Cerebral Amyloid Angiopathy) occurs in the small blood vessels of the brain. Amyloid deposits (toxic proteins) attach themselves to the wall of the blood vessels. This causes recurring brain haemorrhages and infarcts, which can lead to i.e. paralysis, vascular dementia and ultimately death. 1 in 4 people over the age of 60 develops CAA.
The characteristics and development of sporadic CAA is similar to that of Dutchtype CAA, although usually it's onset is later than Dutchtype CAA and the disease course is milder. Sporadic CAA can not be predicted and can only be diagnosed after onset. To diagnose sporadic CAA, doctors use the Boston criteria. There are neurologists who specialize in sporadic CAA. There is no patient association for sporadic CAA. The International CAA Association's website has everything you need to know about CAA.
Because Dutchtype CAA is very similar to sporadic CAA and can be predicted, researchers are interested in following the course of the disease over time. The similarities helped raise attention from pharmaceutical companies, who want to design a treatment for worldwide CAA. A number of those studies are ongoing and the potential for a trial is growing.
There are many similarities between the diseases CAA and Dutchtype CAA. More knowledge of Dutchtype CAA will aid the fight against CAA, which is a far more frequently occurring disease.
Researchers from Leiden (NL), Boston (USA) and Perth (AUS) are working together with the patient assocation, the Dutch CAA Foundation and the pharmaceutical companies Alnylam and Regeneron to study disease trajectory and a possible RNA therapy. Hopefully this therapy will work for both genetic and sporadic CAA.