Hereditary cystatin C amyloid angiopathy
A fatal Icelandic hereditary cerebral amyloid angiopathy causing early brain hemorrhages.
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Hereditary cystatin C amyloid angiopathy is a fatal brain condition passed down in families, seen almost only in Iceland. A faulty cystatin C protein builds up as amyloid in brain arteries, causing repeated strokes. Symptoms, like paralysis or dementia, usually start in the late teens or early twenties, and most die around age 30.
Quick Facts
- Inheritance
- Autosomal dominant
- Geographic concentration
- Iceland, most traced to Breiðafjörður in the northwest
- Average age of death
- Around 30
- Exception to geographic pattern
- One patient in the US with a sporadic mutation
Facts from the source article.
More in Vascular Diseases
Sources
Compiled from Wikipedia and the sources listed below. Text from Wikipedia is available under CC BY-SA 4.0; this entry is adapted from it.
- Wikipedia: Hereditary cystatin C amyloid angiopathy (CC BY-SA 4.0).
- Word definitions: the Codexery glossary, each quoted from its Wikipedia article.
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