D-CAA, also known as the 'Katwijk disease', is a hereditary form of cerebral haemorrhaging.
D-CAA is the acronym for Dutch Cerebral Amyloid Angiopathy. The 'D' stands for 'Dutch type'. In Dutch it is called: erfelijke hersenbloedingen met amyloïdose (accumulation of an unusual protein) - Dutch type. D-CAA finds its origins in Katwijk (a town located on the Dutch North Sea) and is mainly found in a small number of Dutch families and some emigrants in Australia with Dutch ancestral roots.
A distinct trait of the disease HCHWA -D is the accumulation of proteins (amyloids) in the arteries of the brain. This causes cerebral haemorrhaging. The first stroke usually occurs between the ages of 45 and 65, however, incidents have been reported of younger as well as older patients. The severity as well as resulting effects of the haemorrhages can vary greatly. Also, the number of attacks differs per person. D-CAA can be related to dementia.
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The gene responsible for D-CAA was discovered in 1990. The inheritance of the gene is autosomal dominant (non-sex-related). This means that there is a 50% chance that a child will inherit the disorder from a parent who carries the D-CAA gene. At the moment, there are still no possibilities of preventing or curing this disease.
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D-CAA is commonly referred to as 'Katwijk disease'. Two out of three D-CAA affected families live or used to live in, or around, Katwijk. The third family currently resides in Scheveningen. It is possible, though not confirmed, that all three families may have descended from a common ancestor and are therefore distant relatives.