Learn About Hereditary Cerebral Amyloid Angiopathy

What is the definition of Hereditary Cerebral Amyloid Angiopathy?

Hereditary cerebral amyloid angiopathy is a condition characterized by an abnormal buildup of protein clumps called amyloid deposits in the blood vessels in the brain, causing vascular disease (angiopathy). People with hereditary cerebral amyloid angiopathy often have progressive loss of intellectual function (dementia), stroke, and other neurological problems starting in mid-adulthood. Due to neurological decline, this condition is typically fatal in one's sixties, although there is variation depending on the severity of the signs and symptoms. Most affected individuals die within a decade after signs and symptoms first appear, although some people with the disease have survived longer.

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What are the causes of Hereditary Cerebral Amyloid Angiopathy?

Variants (also called mutations) in the APP gene are the most common cause of hereditary cerebral amyloid angiopathy. APP gene variants cause the Dutch, Italian, Arctic, Iowa, Flemish, and Piedmont types of this condition. Variants in the CST3 gene cause the Icelandic type. Familial British and Danish dementia are caused by variants in the ITM2B gene.

How prevalent is Hereditary Cerebral Amyloid Angiopathy?

The prevalence of hereditary cerebral amyloid angiopathy is unknown. The Dutch type is the most common, with over 200 affected individuals reported in the scientific literature.

Is Hereditary Cerebral Amyloid Angiopathy an inherited disorder?

Hereditary cerebral amyloid angiopathy caused by mutations in the APP, CST3, or ITM2B gene is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.

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What are the latest Hereditary Cerebral Amyloid Angiopathy Clinical Trials?
Safety and Efficacy of Remote Ischemic Conditioning in Patients With Cerebral Amyloid Angiopathy-related Intracerebral Hemorrhage:A Multicenter, Randomized, Controlled Study

Summary: Cerebral amyloid angiopathy-related intracerebral (CAAH) hemorrhage is second factor of primary intracerebral hemorrhage. However, no effective prevention and treatment strategies have been established. Remote ischemic conditioning is a neuroprotective strategy. In animal studies,RIC is efficiency in accelerating the absorption of hematoma. Therefore, the investigators plan to carry out this resea...

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COrtical Superficial Siderosis and REcurrent Lobar Intracerebral Hemorrhage in Cerebral Amyloid Angiopathy.

Summary: Cerebral amyloid angiopathy (CAA) is a major cause of lobar intracerebral hemorrhage (ICH) in the elderly with high risk of recurrence. The investigators aim to determine the relationship between cortical superficial siderosis (cSS), a MRI hemorrhagic marker of CAA and the risk of symptomatic ICH recurrence in a multicentric prospective cohort of patients with acute lobar ICH related to CAA. The i...

Who are the sources who wrote this article ?

Published Date: April 11, 2022Published By: National Institutes of Health

What are the Latest Advances for Hereditary Cerebral Amyloid Angiopathy?

There is no recent research available for this condition. Please check back because thousands of new papers are published every week and we strive to find and display the most recent relevant research as soon as it is available.