Amyloid Angiopathy: Causes, Symptoms & Treatment

Amyloid angiopathy is a disease that affects the blood-bearing vessels of the brain. Beta-amyloids are deposited inside the blood vessels, causing the lumen to narrow. In addition, microaneurysms sometimes develop. This results in the risk of the aneurysms bursting and causing bleeding in the brain.

What is amyloid angiopathy?

Amyloid angiopathy is a disease that affects the blood-carrying vessels of the brain. Amyloid angiopathy is referred to by the synonym cerebral amyloid angiopathy or the abbreviation CAA. In 1938, the researcher Scholz described amyloid angiopathy for the first time in patients of advanced age. He discovered the typical deposits of beta-amyloids in the brains of these individuals. Because certain parallels were found in the signs of the disease, amyloid angiopathy was initially categorized as Alzheimer’s disease. However, researchers later found that amyloid angiopathy is a disease in its own right. Thus, amyloid angiopathy does not develop as a result of hypertension, which is the cause of many other brain hemorrhages. Most commonly, amyloid angiopathy is manifested by deposits of beta-amyloids. A large proportion of patients with Alzheimer’s disease suffer from such conditions. In addition, there are several genetically caused types of amyloid angiopathy. These involve genetic mutations affecting, for example, presenilin and the amyloid precursor protein. A gene mutation of cystatin C also occurs in some cases. Basically, amyloid angiopathy and the associated symptoms result from the storage of certain peptides in the blood-bearing vessels of the brain. In most cases, the peptide beta-amyloid is deposited. The deposits accumulate in different areas inside the blood system in the brain. As a result, various stenoses and often microaneurysms develop.

Causes

Amyloid angiopathy develops as a result of the deposition of the peptide beta-amyloid. This substance forms as the amyloid precursor protein breaks down. This requires the enzymes gamma- and betasecretase. In healthy individuals, the metabolism does not produce these peptides. Presumptions assume that primarily nerve cells produce the substance amyloid. The substance accumulates in the neural fluid in patients, so that deposits gradually form in the walls of the cerebral blood vessels or the tissue of the brain. These deposits are known as senile plaques and occur mainly in advanced age. They also occur in association with Alzheimer’s dementia. Certain genetic factors increase the risk of developing amyloid angiopathy. In particular, the allele called ApoE4 predisposes affected individuals to accumulate corresponding deposits of amyloid in blood vessels.

Symptoms, complaints, and signs

Amyloid angiopathy manifests itself primarily in the typical deposits of the peptide beta-amyloid in the blood-bearing vessels in the brain. The substance accumulates particularly in the walls of the vessels. A special amyloid precursor protein is required for the formation of beta-amyloid. This is formed when the precursor substance is divided by enzymatic processes. In this way, the pathogenic beta-amyloid is formed. Such processes occur exclusively in the neuronal tissue of patients. In healthy people, on the other hand, the organism does not produce any fraction of beta-amyloid. The peptide accumulates in the nervous fluid, the so-called cerebrospinal fluid. Characteristic plaque then forms. These deposits in the tissue of the nerves are largely responsible for the development of Alzheimer’s disease.

Diagnosis and progression

The diagnosis of amyloid angiopathy is extremely difficult to make in living patients. Therefore, a reliable diagnosis is often only successful in autopsies of deceased patients. These reveal minimal bleeding into the tissues of the brain with no obvious cause. This allows the conclusion of an amyloid angiopathy. The diagnosis is based on the performance of an MRI examination. Here, hyalinized blood vessels are visible, with loss of smooth muscle cell structures. Clinical diagnosis is based on imaging studies showing isolated or multiple lobar hemorrhages in the brain.These hemorrhages occur mainly at the border between cortex and medulla. In addition, microhemorrhages into the brain are possible, which do not reveal any other cause. Statistical evaluations show that approximately five to twelve percent of intracranial hemorrhages are caused by an amyloid angiopathy. The data apply only to people older than 55 years. People who have amyloid angiopathy and take certain anticoagulants are particularly likely to experience bleeding in the brain.

Complications

Amyloid angiopathy is a relatively serious disease. In the worst cases, severe bleeding in the brain can occur, creating a life-threatening situation for the patient. In most affected individuals, Alzheimer’s disease develops as a result of the amyloid angiopathy. This can develop over time and does not have to occur suddenly. It is difficult for the physician to detect the amyloid angiopathy, which is why a specific treatment is not possible. However, it is possible to identify the bleeding in the brain during an autopsy. Unfortunately, treatment of amyloid angiopathy is not possible. The patient must live with the disease, although a healthy lifestyle can have a positive effect on the disease and alleviate symptoms. Different complications can occur due to Alzheimer’s disease. Most people can no longer live alone due to this diet and are dependent on the support of family or caregivers. Everyday life is severely restricted by the amyloid angiopathy and the quality of life decreases considerably. Unfortunately, it is not possible to treat Alzheimer’s disease completely. The symptoms that occur can only be partially alleviated.

When should you see a doctor?

In the worst case, amyloid angiopathy can cause bleeding in the brain. To avoid these hemorrhages and thus other consequential damages, a doctor should be consulted in any case when symptoms of this disease occur. This can also prevent the premature death of the affected person. Especially in the case of water retention in various regions of the body, a medical examination and treatment should be carried out. In most cases, these are associated with complaints of blood circulation. Likewise, a doctor should also be consulted when the affected person shows the first symptoms and complaints of Alzheimer’s disease. As a rule, the disease cannot be completely cured, but only stopped. Patients suffer from forgetfulness, disturbances in concentration and coordination. Orientation and ordinary thinking and acting can also be limited by amyloid angiopathy. Relatives in particular must pay attention to these symptoms and request an examination if necessary. An examination is also advisable in the case of reduced resilience or constant fatigue. This is especially true if these complaints occur unexpectedly and without any particular reason. It is also possible that amyloid angiopathy may limit the patient’s life expectancy.

Treatment and therapy

Currently, no treatment-related measures are available for the therapy of amyloid angiopathy. Neither causative nor symptomatic approaches to treatment exist. Amyloid angiopathy often accompanies Alzheimer’s disease, requiring special care measures for affected patients. In some cases, individuals receive outpatient care or live in assisted living facilities to help them cope with everyday life with the disease. A generally healthy lifestyle may have a beneficial effect on overall health, thereby improving quality of life despite amyloid angiopathy.

Outlook and prognosis

In many cases, early treatment of amyloid angiopathy is not possible because the symptoms and signs of this disease are gradual in onset and eventually lead to Alzheimer’s disease. The quality of life of the affected person is significantly limited and reduced by the disease, so that it can also lead to difficulties in everyday life for the affected person. An independent cure of the amyloid angiopathy does not usually occur. Furthermore, Alzheimer’s disease enormously restricts the patient’s everyday life, so that the patient may be dependent on the help of other people. It is not uncommon for coordination and concentration to be impaired.Thinking and acting are also disturbed, so that the patient can be a danger to himself or to other people. In many cases, amyloid angiopathy also means that the affected person can no longer feed themselves independently. Unfortunately, direct treatment of amyloid angiopathy is not possible. The symptoms can be limited and alleviated in some cases, but a causal and fundamental treatment of this disease is not possible. The patient’s life expectancy may be reduced by amyloid angiopathy as well.

Prevention

No preventive measures of amyloid angiopathy are yet available and tested. The disease occurs more frequently in older age as a result of a metabolic disorder. However, the exact causes of amyloid angiopathy have not been researched. To date, there are no effective ways to influence the corresponding metabolic processes that contribute significantly to the development of amyloid angiopathy.

Follow-up

The options for aftercare are usually very limited in amyloid angiopathy. Affected individuals always rely on medical treatment to relieve symptoms and prevent further complications. Treatment by means of self-help cannot be applied in this case either. Possibly, the amyloid angiopathy also leads to a reduced life expectancy of the affected person. The affected persons are mainly dependent on a permanent care in their life, because the disease cannot be treated completely. First and foremost, loving and intensive care and support from one’s own relatives or friends has a very positive effect on the further course of the disease. A healthy lifestyle with a healthy diet can also have a positive effect on the course of amyloid angiopathy. Affected persons should refrain from taking nicotine and alcohol. Affected persons must be intensively encouraged to counteract the disease. This is the only way to preserve memory. It is recommended to visit the patients frequently and to have a lot of contact with the affected persons. Contact with other amyloid angiopathy patients may also be useful.

Here’s what you can do yourself

The disease offers few self-help options for the affected person. In a large number of cases, diagnosis does not occur until acute stages of life or after death. The development of impairments are hardly or not at all perceived by the affected person in everyday life. Therefore, the development as well as the progression of the disease of amyloid angiopathy often remain unnoticed for a long time. Basically, an improvement in health can be achieved by structuring the general lifestyle. Through diet, lifestyle and and the design of life based on physical needs, a significant improvement in well-being can be achieved. Although these measures contribute to an increase in the quality of life in everyday life, it cannot be excluded that the amyloid angiopathy develops further. Scientists found that a healthy lifestyle has a positive influence on the progression of the disease. Symptoms are alleviated and the progress of the disease is significantly curbed. Therefore, special attention should be paid to a balanced diet. This stabilizes and supports the body’s own defense system. In addition, it contributes significantly to minimizing complications. The form of nutrition in combination with good sleep hygiene and the avoidance of harmful substances such as alcohol and nicotine reduces the likelihood of severe disease progression.