What is the definition of Cerebral Amyloid 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.

What are the alternative names for Cerebral Amyloid Angiopathy?

Amyloidosis - cerebral; CAA; Congophilic angiopathy

What are the causes for Cerebral Amyloid Angiopathy?

People with CAA have deposits of amyloid protein in the walls of blood vessels in the brain. The protein is usually not deposited anywhere else in the body.

The major risk factor is increasing age. CAA is more often seen in people older than 55. Sometimes, it is passed down through families.

What are the symptoms for Cerebral Amyloid Angiopathy?

CAA can cause bleeding into the brain. Bleeding often occurs in the outer parts of the brain, called the cortex, 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:

  • Drowsiness
  • Headache (usually in a certain part of the head)
  • Nervous system changes that may start suddenly, including confusion, delirium, double vision, decreased vision, sensation changes, speech problems, weakness, or paralysis
  • Seizures
  • Stupor or coma (rarely)
  • Vomiting

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

What are the current treatments for Cerebral Amyloid Angiopathy?

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.

What is the outlook (prognosis) for Cerebral Amyloid Angiopathy?

The disorder slowly gets worse.

What are the possible complications for Cerebral Amyloid Angiopathy?

Complications of CAA may include:

  • Dementia
  • Hydrocephalus (rarely)
  • Seizures
  • Repeated episodes of bleeding in the brain

When should I contact a medical professional for Cerebral Amyloid Angiopathy?

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.

Amyloidosis
Arteries

REFERENCES

Charidimou A, Boulouis G, Gurol ME, et al. Emerging concepts in sporadic cerebral amyloid angiopathy. Brain. 2017;140(7):1829-1850. PMID: 28334869 pubmed.ncbi.nlm.nih.gov/28334869/.

Greenberg SM, Charidimou A. Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke. 2018;49(2):491-497. PMID: 29335334 pubmed.ncbi.nlm.nih.gov/29335334/.

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.