What is CAA?

What is CAA?
The disease CAA (Cerebral Amyloid Angiopathy) occurs in the small blood vessels of the brain. Amyloid (toxic protein) accumulates in the wall of these vessels. This causes repeated cerebral haemorrhages and infarctions, which lead to paralysis, dementia and eventually death. One in four people over the age of sixty get CAA. And this number is increasing.

Professor Greenberg explains CAA, click here.

HCHWA-D (full name: Hereditary Cerebral Haemorrhages with Amyloidosis – Dutch type) is an inherited form of CAA that, unlike CAA, can be determined with certainty during life. This variant is revealed relatively early in life, around the age of 45. It was first identified in Katwijk, Holland, which is why the condition is commonly referred to as the ‘Katwijk disease’. Children of hereditary carriers have a fifty percent chance of developing the disease. Research on these families could not only result in treatment of this rare hereditary variant of CAA, but could also lead to the key for therapy for the much more common, non-hereditary variant of the disease.

CAA is not a new disease. The lack of awareness has to do with fact that, for many years, the disease could not be diagnosed during life. Only recently has it been discovered that brain scans can diagnose symptoms of the disease. Even though the indications do not offer certainty, they have led to the awareness that CAA is a common disease. The full extent of the effects of CAA has also recently become known. CAA is the cause of a quarter of all cerebral haemorrhages in older people, and the disease is increasingly recognised as a major cause of loss of brain functions and dementia in the elderly. HCHWA-D was discovered in the 1980s and recognised years later as a hereditary variant of CAA. Watch a film about CAA here.

Patient Association
Currently there isn’t a global CAA patient association. However, the carriers of the hereditary Dutch CAA variant have joined in the HCHWA-D Association: www.hchwa-d.nl

You can contact the telephone consultation line (+31 (0)6-22062560) of the HCHWA-d Care Desk on Wednesday mornings from 9:30 am to 12:00 pm. This Care Desk provides advice and can point you in the right direction to find the support you need.

You can also email your questions to the Care Desk. The email is checked daily: zorgloket@hchwa-d.nl


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