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Diseases

Genetic and Rare Diseases Information Center (GARD)

Hereditary cerebral hemorrhage with amyloidosis


Other Names for this Disease
  • Cerebral amyloid angiopathy
  • HCHWA
  • CAA, familial
  • Cerebral amyloid angiopathy, familial
  • cerebral amyloid angiopathy, genetic
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My father as well as several other family members had cerebral amyloid angiopathy (CAA).  What are my chances of having the condition?  What is the difference between familial CAA and Dutch type CAA?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is hereditary cerebral hemorrhage with amyloidosis? 

Hereditary cerebral hemorrhage with amyloidosis (HCHWA) is a neurological condition in which an abnormal protein (amyloid) builds up in the walls of the arteries of the brain (and less frequently, veins). This process is known as amyloid deposition, which can lead to strokes, seizures, neurological deficits, cognitive decline, and dementia. Symptoms usually present before the 5th decade of life.[1] There are many different ways of classifying the types of HCHWA based on the underlying genetic changes, proteins involved, signs and symptoms, and the regions in which they were first described. Based on the region in which they were first described, the subtypes include:[1][2]
All types of HCHWA currently described are inherited in an autosomal dominant manner.[2] There is no cure for HCHWA, however, antihypertensive therapy is typically recommended.[3][1]
Last updated: 7/19/2016

What is the difference between "familial" cerebral amyloid angiopathy and cerebral amyloid angiopathy, Dutch type?

"Familial" cerebral amyloid angiopathy is another term often used to refer to hereditary cerebral hemorrhage with amyloidosis. Hereditary cerebral hemorrhage with amyloidosis is the term more commonly used in the medical literature. Hereditary cerebral hemorrhage with amyloidosis, Dutch type is a specific form of hereditary cerebral hemorrhage with amyloidosis that is caused by a mutation (genetic change) in the APP (amyloid precursor protein) gene. There is at least one other form of hereditary cerebral hemorrhage with amyloidosis: the "Icelandic type," which is due to a mutation in the cystatin C gene.[4]
Last updated: 7/19/2013

Since I have a family history of hereditary cerebral hemorrhage with amyloidosis, what are the chances that I inherited the condition?

To find out your chances of having hereditary cerebral hemorrhage with amyloidosis, you may want to speak with a genetics professional. A genetics professionl can review your medical and family history in order to provide you with your specific risks. To learn more about genetic consultations, click here.
Last updated: 7/19/2013

How can I find a genetics professional in my area?

Genetics clinics are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about genetic consultations is available from Genetics Home Reference. To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral.

The following online resources can help you find a genetics professional in your community:
Last updated: 5/18/2016

References
Other Names for this Disease
  • Cerebral amyloid angiopathy
  • HCHWA
  • CAA, familial
  • Cerebral amyloid angiopathy, familial
  • cerebral amyloid angiopathy, genetic
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.