Moyamoya disease is a disease that affects the vessels of the brain. As a result of a disease vessels in a brain area spontaneously close. The occlusion takes place over a longer period of time by a fibrous transformation in the area of ​​the brain base. Often the rebuilding takes place at the internal carotid artery.

What is a Moyamoya disease?

Moyamoya disease is most common in Japan. According to current medical findings, the disease is caused by genetic mutations on the 17th chromosome. In addition to the genetic and thus congenital form of Moyamoya disease also exists an acquired manifestation of the disease.

This is shown, for example, in connection with Down syndrome or arteriosclerosis and is also known as Moyamoya syndrome. The name of the Moyamoya disease comes from the Japanese language. Moyamoya here means 'cloud of smoke' and refers to the tiny collateral vessels, which form as a result of stenoses and remind in the form of smoke in the angiogram.

causes

The causes of Moyamoya disease lie in the vast majority of cases in genetic mutations. The gene mutation occurs on chromosome 17 at a specific gene locus. The exact prevalence of Moyamoya disease is not yet known.

The Moyamoya disease is determined by the affected people from birth and develops in the course of life. The disease progresses slowly, but continuously, and leads to occlusion of vessels in the brain, which consequently have an appearance in the angiogram, such as smoke or fog.

Symptoms, complaints & signs

The Moyamoya disease is associated with a closure of vessels in the brain. The blood vessels initially narrow over a relatively long period of time. The progressive narrowing of the vessels eventually leads to a complete occlusion of the arteries. In most cases, the arteria cerebri media and the internal carotid artery are affected by the pathological changes.

As a result of the closed vessels, an increasing anemia develops in the brain of the diseased persons. There is a risk of ischemic attacks and strokes. To compensate for the occluded vessels, numerous tiny blood vessels are formed. These vessels appear in imaging as side-by-side or smoky entities.

In Europe, the Moyamoya disease is relatively rare. Much more often it shows in Asia and here especially in Japan. Moyamoya disease is particularly prevalent in children between the ages of two and ten and between the ages of 30 and 40 years.

As a result of Moyamoya disease and occlusion of cerebral blood vessels, patients are at risk of brain infarction and cerebral hemorrhage. As a result, doctors often discover Moyamoya disease in the context of such complications.

Diagnosis & disease course

In most cases, the diagnosis of Moyamoya disease occurs only when the disease is characterized by typical complications. These include primarily strokes and brain bleeding. Because in many patients, only after such incidents corresponding investigations, which eventually point to a Moyamoya disease.

Rarely, does a diagnosis of moyamoya disease occur before acute emergencies, such as a stroke, occur. The diagnosis of Moyamoya disease usually occurs in specialized centers that are equipped with the necessary brain imaging technology. First, there is an anamnesis with the person affected by the Moyamoya disease, who describes his symptoms and any complications.

Important is always the beginning of the disease symptoms of Moyamoya disease. The clinical investigation relies mainly on imaging techniques. The focus is on the patient's brain. Mostly an MRI examination and an angiography are used. Patients usually receive special contrast media prior to imaging. As a result, the tiny vessels and a reduced supply of blood and oxygen in the brain are detectable.

complications

Since the Moyamoya disease, especially the brain is affected by the symptoms, this disease can have a very negative impact on the life and everyday life of the person affected. As a rule, a stroke occurs in the patient at an early stage, which can lead to various irreversible sequelae. Not infrequently, the patients then suffer from disorders of sensitivity and paralysis.

This limits the individual's motor and physical abilities so that the person concerned may even need the help of other people in everyday life. Without treatment, the Moyamoya disease can also lead to death. It is not possible to treat the Moyamoya disease causally.

However, the symptoms and complications can be limited with the help of medication. Furthermore, in most cases, those affected are dependent on regular examinations to avoid consequential damage. In severe cases, a bypass can also be placed.

A healthy diet and an attentive lifestyle have a very positive effect on the disease and can prevent further complications. In general, however, the life expectancy of the patient is significantly limited by the Moyamoya disease.

When should you go to the doctor?

Moyamoya disease is a serious disease that needs immediate medical attention in any case. People who experience periodic headaches, impaired consciousness and other unusual symptoms should seek medical advice promptly. The doctor can diagnose any Moyamoya syndrome and then initiate treatment directly. If this happens early, serious complications can often be avoided. Lack of treatment can lead to strokes and other health consequences due to narrowing of the cerebral vessels.

At the latest after a stroke or a cerebral hemorrhage a doctor must make a diagnosis. Affected persons must then be treated in a special clinic and closely monitored. Moyamoya disease is treated by neurologists and internists. Because it is a serious illness that often ends in death, those affected should also seek treatment from a therapist. If there is a suspicion of a new vascular occlusion, the responsible doctor must be informed immediately or the nearest hospital must be visited immediately.

Treatment & Therapy

The treatment of Moyamoya disease is symptomatic only, as treatment of the causes of the disease is not feasible. Especially important is a drug therapy of people affected by the Moyamoya disease. The people usually take so-called anticoagulants.

In addition, encephalomyosynangiosis and encephaloduroarteriosynangiosis are suitable as therapeutic measures. It is also possible to treat the Moyamoya disease by means of surgical procedures. The patients receive a neurosurgical bypass as part of inpatient interventions.

This form of therapy has been relatively successful so far. During surgery, the doctors connect the cerebral and temporal artery. It is also of great importance in the successful symptomatic treatment of Moyamoya disease that patients minimize as many personal risk factors as possible in their own lifestyle.

These include, for example, overweight and hypertension reduce and give up smoking. In principle, a regular and permanent medical check of patients is required to monitor the progression of the disease and to intervene as quickly as possible targeted by means of appropriate therapeutic measures.

Outlook & Forecast

Moyamoya disease can be treated well. However, little is known about the long-term course. Cases are known in which no complaints occur. In other cases, there are strokes and circulatory disorders of the brain. The course is based on the age at which the symptoms occur and how strongly they are pronounced.

Left untreated, the condition will be unfavorable as severe neurological deficits develop. First and foremost, there is a high risk of stroke, with a stroke resulting in serious disability or death. Through a direct or indirect bypass method, in which the blood circulation in the brain is promoted, the risk of stroke is significantly reduced. Around 60 percent of all patients are symptom free after the procedure.

Physiotherapy, speech therapy and occupational therapy can further improve the prognosis. The responsible neurologist can make a prognosis based on the symptom picture. However, this must be regularly adjusted, especially in repeated circulatory disorders and other complications. Possible comorbidities, as they occur especially in severe cerebrovascular malformations worsen the prognosis. High-risk patients achieve an optimal prognosis through early diagnosis and treatment.

prevention

The Moyamoya disease is genetically caused in the affected people and thus determined from birth. For this reason, the disease can not be causally prevented. However, medicine is researching ways to effectively prevent genetic disorders such as Moyamoya's disease.

aftercare

Since Moyamoya disease is usually a hereditary disease, it can not be completely cured. Therefore, the person concerned should consult a doctor at the first symptoms and signs of the disease to prevent the occurrence of further complications and complaints. The sooner a doctor is visited, the better is usually the further course of the disease.

If there is a wish for a child, a genetic examination and counseling should be made to prevent the recurrence of the disease in the children. Most patients are dependent on the use of various medications in the disease. The patient should always pay attention to a regular intake and also to a correct dosage of the medicines.

In case of questions or ambiguity, always consult a doctor first. In general, a healthy lifestyle should also be considered, avoiding obesity. The person concerned should also refrain from alcohol and smoking. Eventually, the Moyamoya disease reduces the life expectancy of the patient.

You can do that yourself

The Moyamoya disease can be treated so far only symptomatically. The drug therapy can be supported by those affected by a number of measures. Individual symptoms such as obesity and high blood pressure can be alleviated by changing the lifestyle.

Basically, patients should maintain a healthy lifestyle and pay attention to a balanced diet. If sport is regularly exercised at the same time, the typical symptoms can already be greatly reduced. Stimulants such as cigarettes, alcohol or coffee should be avoided.

Since the disease exists from birth, a diagnosis can be made early. Parents who notice the characteristic symptoms of their child should consult a doctor. Early diagnosis and treatment improves treatment options. On the other hand, possible risk factors can be ruled out from the outset. Nevertheless, Moyamoya patients need permanent medical control. Since the disease is a great burden for all involved, an accompanying therapy for the parents and for the affected child is always recommended.

After surgery, the patient primarily requires bed warmth and protection. In addition, further visits to the doctor are indicated, in which the state of health of the patient can be checked and any complications can be detected early.

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