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Cerebral amyloid angiopathy

Amyloidosis - cerebral; CAA

 

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.

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 bleeding 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. Sometimes, patients develop 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. Such immediate symptoms include:

  • Drowsiness
  • Headache (usually in a specific part of the head)
  • Neurologic changes that may start suddenly, including confusion, deliriumdouble 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: Elsevier Saunders; 2012:chap 51B.

Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007;68:1411-1416. PMID: 17452586 www.ncbi.nlm.nih.gov/pubmed/17452586.

Zivin JA. Hemorrhagic cerebrovascular disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 415.

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    Arteries of the brain

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    Tests for Cerebral amyloid angiopathy

     
       

      Review Date: 7/27/2014

      Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper University Hospital, Camden, NJ. 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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