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Cerebral amyloid angiopathy

Amyloidosis - cerebral; CAA; Congophilic angiopathy

Cerebral amyloid angiopathy (CAA) is a condition in which proteins called amyloid build up on the walls of the arteries in the brain. CAA increases the risk for stroke caused by bleeding and dementia.

Causes

The cause of CAA is unknown. Sometimes, it may be passed down through families.

People with CAA 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 people older than 55.

Symptoms

CAA can cause bleeding into the brain. Bleeding often occurs 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. Some people have gradual memory problems. When a CT scan is done, there are signs that they have had bleeding in the brain that they may not have realized.

If there is a lot of bleeding, immediate symptoms occur and resemble a stroke. These symptoms include:

If bleeding is not severe or widespread, symptoms can include:

  • Episodes of confusion
  • Headaches that come and go
  • Loss of mental function (dementia)
  • Weakness or unusual sensations that come and go, and involve smaller areas
  • Seizures

Exams and Tests

CAA is hard 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 normal if the bleed is small. There may be some brain function changes. It is important for the doctor to ask detailed questions about the symptoms and medical history. The symptoms and results of the physical exam and any imaging tests may cause the doctor to suspect CAA.

Imaging tests of the head that may be done include:

  • CT scan or MRI scan to check for bleeding in the brain
  • MRA scan to check for large bleeds and rule out other causes of bleeding
  • PET scan to check for amyloid deposits in the brain

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.

Sometimes, medicines that help improve memory, such as those for Alzheimer disease, are used.

Seizures, also called amyloid spells, may be treated with anti-seizure drugs.

Outlook (Prognosis)

The disorder slowly gets worse.

Possible Complications

Complications of CAA may include:

When to Contact a Medical Professional

Go to the emergency room or call the local emergency number (such as 911) if you have sudden loss of movement, sensation, vision, or speech.

References

Kase CS, Shoamanesh A. Intracerebral hemorrhage. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 66.

Yamada M. Cerebral amyloid angiopathy: emerging concepts. J Stroke. 2015;17(1):17-30. PMID: 25692104 www.ncbi.nlm.nih.gov/pubmed/25692104.

    • Amyloidosis on the fingers

      Amyloidosis on the fingers - illustration

      Amyloidosis refers to the extracellular deposition of a protein called amyloid. This protein deposition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging, or may occur with several other conditions. In this picture, we see how amyloidosis can affect the skin as nodular deposits on the fingers.

      Amyloidosis on the fingers

      illustration

    • Arteries of the brain

      Arteries of the brain - illustration

      The internal carotid arteries and the vertebral arteries supply blood to the brain.

      Arteries of the brain

      illustration

      • Amyloidosis on the fingers

        Amyloidosis on the fingers - illustration

        Amyloidosis refers to the extracellular deposition of a protein called amyloid. This protein deposition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging, or may occur with several other conditions. In this picture, we see how amyloidosis can affect the skin as nodular deposits on the fingers.

        Amyloidosis on the fingers

        illustration

      • Arteries of the brain

        Arteries of the brain - illustration

        The internal carotid arteries and the vertebral arteries supply blood to the brain.

        Arteries of the brain

        illustration

      Review Date: 5/30/2016

      Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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