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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:

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 malformationor 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.

ComplicationsCalling 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.

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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, 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:

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
Signs 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 malformationor 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).

Expectations (prognosis)

The disorder slowly gets worse but varies from person to person.

ComplicationsCalling 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;68:1411-1416.

Zivin JA. Hemorrhagic cerebrovascular disease. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 432.

Reviewed By

Review Date: 08/27/2010

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.

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Related questions

What is another name for Cerebral amyloid angiopathy?

Cerebral amyloid angiopathy (CAA) is also known as congophilic angiopathy or cerebrovascular amyloidosis.


What is the average age of onset for Cerebral amyloid angiopathy?

The average age of onset for cerebral amyloid angiopathy is around 60-70 years old. However, onset can occur earlier in some cases, particularly in individuals with genetic predispositions or underlying health conditions.


What is the most common type of Cerebral amyloid angiopathy?

The most common form of CAA is the sporadic form associated with aging. This type of CAA usually causes lobar hemorrhage, which may recur in different lobes of the brain.


Is Cerebral amyloid angiopathy a hereditary disease?

No. The most common cause of intracerebral hemorrhage is high blood pressure (hypertension).


What causes sporadic Cerebral amyloid angiopathy?

CAA may be found during an autopsy in over one-third of persons over age 60, even though they may not have had brain hemorrhage, stroke, or other manifestations of the disease during life.


How do you spell CAA?

The acronym CAA may stand for Canadian Automobile Association Cerebral Amyloid Angiopathy (brain blood vessel disease) Civil Aviation Authority (UK) Civil Aeronautics Administration (Taiwan) Clean Air Act (US 1963) Colonial Athletic Association (NCAA schools)


What form of CAA is Flemish type of hereditary cerebral hemorrhage with amyloidosis?

autosomal dominant, with a mutation involving the amyloid precursor protein. Symptoms include brain hemorrhage or dementia.


What is an amyloid?

An amyloid is a waxy compound of protein and polysaccharides which is found deposited in tissues in amyloidosis.


What is amylodiosis?

A disorder marked by deposition of amyloid in the body. a rare disease which causes the build-up of amyloid, a protein, & starch, it can get in tissues and organs.


Signs of amyloid proteins can be indicated by?

Blood and urine test


How does amyloidosis occur?

Amyloid proteins are manufactured by malfunctioning bone marrow. Amyloidosis, which occurs when accumulated amyloid deposits impair normal body function, can cause organ failure or death.


What has the author Dankwart Stiller written?

Dankwart Stiller has written: 'Histochemistry of amyloid' -- subject(s): Amyloid, Amyloidosis, Analysis, Cytologic Diagnosis, Electron microscope, Histochemistry, Ultrastructure (Biology)