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Cerebral amyloid angiopathy
Definition
Cerebral amyloid angiopathy is a neurological condition in which proteins called amyloid build up on the walls of the arteries in the brain. The condition increases the risk of hemorrhagic stroke and dementia.
Alternative Names
Amyloidosis - cerebral; CAA
Causes
The cause of cerebral amyloid angiopathy is unknown. Sometimes, it may be passed down through families.
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. It is more often seen 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 in a specific part of the head)
- Neurologic changes that may start suddenly, including:
- Confusion, delirium
- Double vision, decreased vision
- Sensation changes
- Speech difficulties
- Weakness or paralysis
- Seizures
- Stupor or coma (rarely)
- Vomiting
Other 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
Exams and Tests
Cerebral amyloid angiopathy is difficult to diagnose with certainty without a sample of brain tissue. This is usually done after death or when a biopsy of the blood vessels of the brain is done.
A physical exam can be relatively normal if you have a small bleed, but you may show some brain function changes. It is important for the doctor to ask detailed questions about your medical history. Your symptoms and 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.
Another type of MRI scan can help show tiny areas where blood has escaped from blood vessels into brain tissue.
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.
Seizures, sometimes called “amyloid spells,” may be treated with anticonvulsants such as phenytoin (Dilantin) and carbamazepine (Tegretol).
Outlook (Prognosis)
The disorder slowly gets worse but varies from person to person.
Possible Complications
- Dementia
- Hydrocephalus (rarely)
- Seizures
- Repeated episodes of bleeding in the brain
When to Contact a Medical Professional
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
Kase CS. Vascular diseases of the nervous system: intracerebral hemorrhage. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley’s Neurology in Clinical Practice. 6th ed. Philadelphia, Pa: Saunders Elsevier;2012:chap 51B.
Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007;68:1411-1416.
Zivin JA. Hemorrhagic cerebrovascular disease. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 415.
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; 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.
