National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Hereditary cerebral hemorrhage with amyloidosis



Other Names:
Cerebral amyloid angiopathy; HCHWA; CAA, familial; Cerebral amyloid angiopathy; HCHWA; CAA, familial; Cerebral amyloid angiopathy, familial; cerebral amyloid angiopathy, genetic See More
This disease is grouped under:

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

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance 0000006
Cerebellar hemorrhage 0011695
Cerebral amyloid angiopathy 0011970
Cerebral ischemia
Disruption of blood oxygen supply to brain
0002637
Dementia
Dementia, progressive
Progressive dementia
[ more ]
0000726
Recurrent cerebral hemorrhage 0004968
Stroke 0001297
Tortuous cerebral arteries
Twisted cerebral arteries
0004938
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Last updated: 7/1/2020

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

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources


Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include all other conditions that could cause lobar intracerebral hemorrhage such as coagulopathies, vasculitis, intravascular large B-cell lymphoma (see these terms), CNS neoplasms, cavernous malformations, cerebral vascular malformation and antecedent trauma
Visit the Orphanet disease page for more information.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease


These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • Amyloidosis Awareness is an illustrated booklet for patients and physicians developed by Amyloidosis Support Groups Inc. Versions of the booklet are also available in Spanish and Portuguese.
  • Genetics Home Reference (GHR) contains information on Hereditary cerebral hemorrhage with amyloidosis. This website is maintained by the National Library of Medicine.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hereditary cerebral hemorrhage with amyloidosis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • Where can I find information about the symptoms of hereditary cerebral hemorrhage with amyloidosis - Dutch type? See answer

  • 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? See answer



  1. Dr Joost HAAN, Dr G.M. [Gisela] TERWINDT. Hereditary cerebral hemorrhage with amyloidosis. Orphanet. February 2013; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=85458.
  2. hereditary cerebral amyloid angiopathy. Genetics Home Reference. May 2012; https://ghr.nlm.nih.gov/condition/hereditary-cerebral-amyloid-angiopathy.
  3. Menon RS. Cerebral Amyloid Angiopathy. Medscape. April 25, 2016; http://emedicine.medscape.com/article/1162720-overview.