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Cerebral amyloid angiopathy
Definition
Cerebral amyloid angiopathy is a neurological condition in which amyloid protein builds up on the walls of the arteries in the brain. The condition increases one's risk of hemorrhagic stroke.
Alternative Names
Amyloidosis - cerebral; CAA
Causes, incidence, and risk factors
The cause of cerebral amyloid angiopathy is unknown. Persons with this condition have deposits of amyloid protein in the walls of the brain arteries. The protein is usually not deposited anywhere else in the body.
The major risk factor is increasing age. The rate of the condition is much greater in those older than 60.
Symptoms
Cerebral amyloid angiopathy can cause bleeding into the brain, usually in the outer parts of the brain, called the lobes, and not the deep areas. Symptoms occur because bleeding in the brain harms brain tissue. If there is a lot of bleeding, immediate symptoms occur and resemble a stroke. Such symptoms include:
- Drowsiness
- Headache (usually on a specific part of the head)
- Neurologic changes that may start suddenly, including:
- Confusion, delirium
- Double vision, decreased vision
- Sensation changes or loss of sensation in an area
- Speech difficulties
- Weakness or paralysis
- Seizures
- Stupor or coma (rarely)
- Vomiting
Some bleeds are very small and occur in different parts of the brain over many months. Symptoms can include:
- Episodes of confusion
- Headaches that come and go
- Loss of mental function (dementia)
- Sensation changes (unusual sensations) that come and go
- Seizures
Signs and tests
Cerebral amyloid angiopathy is difficult to diagnose until after death, when a biopsy of the blood vessels of the brain can be done.
A physical exam can be relatively normal if you have a small bleed, but you may show some brain function changes. Your symptoms and the the results of your physical exam and any imaging tests may cause your doctor to suspect this problem.
A CT scan or MRI of the head may show:
- Bleeding in the brain
- Signs of prior bleeding in the brain
Magnetic resonance angiography (MRA) of the brain can help with the diagnosis of large bleeds and may be used to rule out arteriovenous malformation or aneurysm as the cause of the bleed.
Treatment
There is no known effective treatment. The goal of treatment is to relieve symptoms. In some cases, rehabilitation is needed for weakness or clumsiness. This can include physical, occupational, or speech therapy.
Occasionally, some patients may benefit from medications that help improve memory, such as those used to treat Alzheimer's disease.
Expectations (prognosis)
The disorder slowly gets worse but varies from person to person.
Complications
- Dementia
- Hydrocephalus (rarely)
- Seizures
- Repeated episodes of bleeding in the brain
Calling your health care provider
Go to the emergency room or call the local emergency number (such as 911) if you have a sudden loss of movement, sensation, vision, or speech.
References
Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007 Apr 24;68(17):1411-6.
Reviewed By: Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
