What Is Cerebral Amyloid Angiopathy?

Cerebral amyloid angiopathy is a disorder in which a protein called amyloid accumulates in the brain, inside artery walls. This can lead to weakening of the arteries, which may burst and bleed, giving rise to the symptoms of a stroke. Cerebral amyloid angiopathy is associated with dementia, although there is no proof that either condition causes the other. There is no cure and the disease usually progressively worsens, although treatment can be given for the symptoms and complications. Other names for the condition include cerebrovascular amyloidosis and congophilic angiopathy.

Although research into the condition is ongoing, cerebral amyloid angiopathy causes remain unknown. The disorder is not associated with the disease known as primary amyloidosis, where amyloid protein accumulates in a number of different areas throughout the body. In cerebral amyloid angiopathy, the buildup of abnormal proteins only occurs inside medium and small-sized cerebral arteries in the brain. Occasionally, amyloid is also deposited in cerebral veins.

For some patients, there may be no cerebral amyloid angiopathy symptoms. When symptoms occur, most often they result from a bleed into the brain. The amyloid proteins deposited in artery walls damage them, causing weakened areas that bulge under the pressure of blood flowing through the vessel. This leaves the arteries at greater risk of rupturing. Sometimes, a bleed is only small and may resolve on its own, but a more substantial bleed may require surgery to remove accumulated blood, while a very large bleed may be fatal.

In patients with cerebral amyloid angiopathy, the symptoms of a bleed into the brain vary according to its severity. They could include headaches, difficulty thinking, fits, and problems such as weakness, numbness or odd sensations in some parts of the body. A diagnosis of bleeding may be made using CT scans or MRI scans, but a definite diagnosis of cerebral amyloid angiopathy depends on finding amyloid proteins. These could be discovered located in a blood clot after its surgical removal from the brain. As with some other brain disorders, the only certain diagnosis occurs at post-mortem, when the arteries of the brain can be examined directly.

While there is no curative cerebral amyloid angiopathy treatment, people with symptoms of dementia may find that some of the medications used to treat Alzheimer's could help with memory problems. Patients experiencing seizures may benefit from drugs used to treat epilepsy. Speech therapy and physiotherapy can be used to manage difficulties resulting from weakness and coordination loss.