ABeta amyloidosis, Iowa type

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ORPHA:324708OMIM:605714E85.4+I68.0*
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Overview

ABeta amyloidosis, Iowa type (also called Iowa-type hereditary cerebral amyloid angiopathy or Iowa-type CAA) is an extremely rare inherited brain disease caused by a specific change in the APP gene, which provides instructions for making amyloid precursor protein. In this condition, an abnormal form of amyloid-beta protein builds up in the walls of small blood vessels in the brain. This buildup weakens the blood vessel walls and can lead to repeated strokes, bleeding in the brain, and progressive loss of mental abilities (dementia). The disease was first identified in a family from Iowa, which is how it got its name. Symptoms typically begin in middle adulthood, often between the ages of 50 and 65. Early signs may include memory problems, difficulty with thinking and reasoning, personality changes, and problems with speech or language. As the disease progresses, patients may experience seizures, difficulty walking, and increasing dependence on others for daily activities. Some patients develop severe strokes caused by bleeding within the brain tissue. There is currently no cure for Iowa-type ABeta amyloidosis. Treatment focuses on managing symptoms and preventing complications. Doctors may prescribe medications to control seizures and manage blood pressure. Avoiding blood-thinning medications is often recommended because of the increased risk of brain bleeding. Supportive care, including physical therapy, occupational therapy, and speech therapy, can help maintain quality of life. Research into amyloid-targeting therapies is ongoing, but no disease-modifying treatment has been approved specifically for this condition.

Also known as:

Key symptoms:

Memory lossProgressive dementiaDifficulty with thinking and reasoningPersonality and behavior changesDifficulty speaking or understanding speechSeizuresStrokes caused by brain bleedingHeadachesDifficulty walking or loss of coordinationVision problemsWeakness on one side of the bodyConfusion and disorientationLoss of independence over time

Clinical phenotype terms (9)— hover any for plain English
Abnormal cerebral vascular morphologyHP:0100659
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for ABeta amyloidosis, Iowa type.

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No actively recruiting trials found for ABeta amyloidosis, Iowa type at this time.

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No specialists are currently listed for ABeta amyloidosis, Iowa type.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to ABeta amyloidosis, Iowa type.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What stage is the disease at based on my current symptoms and brain imaging?,Are there any medications I should absolutely avoid because of the bleeding risk?,Should my family members be tested for the APP gene mutation?,Are there any clinical trials I might be eligible for?,What emergency signs should I and my family watch for?,What support services are available to help with daily living and caregiving?,How often should I have follow-up brain imaging to monitor for new bleeds?

Common questions about ABeta amyloidosis, Iowa type

What is ABeta amyloidosis, Iowa type?

ABeta amyloidosis, Iowa type (also called Iowa-type hereditary cerebral amyloid angiopathy or Iowa-type CAA) is an extremely rare inherited brain disease caused by a specific change in the APP gene, which provides instructions for making amyloid precursor protein. In this condition, an abnormal form of amyloid-beta protein builds up in the walls of small blood vessels in the brain. This buildup weakens the blood vessel walls and can lead to repeated strokes, bleeding in the brain, and progressive loss of mental abilities (dementia). The disease was first identified in a family from Iowa, which

How is ABeta amyloidosis, Iowa type inherited?

ABeta amyloidosis, Iowa type follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does ABeta amyloidosis, Iowa type typically begin?

Typical onset of ABeta amyloidosis, Iowa type is adult. Age of onset can vary across affected individuals.