Introduction

Moyamoya is a Japanese word which means ‘puff of smoke’. This term is assigned to the condition based on the generated image of the blood vessels, in the head and the neck region (also known as arteries), in a diagnostic procedure known as angiography, as the impacted arteries look like moyamoya, i.e. a puff of smoke or cloud.

Moyamoya disease is a medical condition in which patients suffer from the narrowing of the arteries, followed by their blockage, which can cause life-threatening complications such as stroke and seizures. 

Moyamoya disease persists for a long period and worsens over time. Moyamoya often attacks children aged 10-14 or people in their 40s. People assigned female at birth (AFAB) and asian people are more prone to suffer from moyamoya disease.^1,2,3,4

Signs and symptoms of moyamoya disease

• An intense headache that occurs in a sudden manner
• Impaired vision - blurriness
• Issues in maintaining balance
• Difficulty speaking
• One side of the body seems to be numb and weak
• Concentration level decreases⁴

Treatment of moyamoya disease

The complications from moyamoya disease can be life-threatening; therefore, it is important for the treatment approach to be direct. One of the most effective ways to treat the disease is to remove the cluster of narrowed blood vessels surgically.

However, moyamoya is still considered to be non-curable. Unfortunately, surgery is not possible in all cases and is considered a temporary improvement of the condition.. Thus, the main treatment path is focused on maintaining the blood flow to the brain, whereby the risk of stroke is minimised, and other complications can be kept away.^2,3

Treatment of the moyamoya disease requires management of a few other interdependent conditions, like high blood pressure, diabetes, and high cholesterol. A crucial point to be considered and taken care of is other factors which can contribute in narrowing the blood vessels further.⁴

In order to minimise the effects of the disease, lifestyle changes are of utmost importance. Here is the list of the factors which can increase the chance of  the person’s symptoms worsening and developing complications:

• Hypertension (high blood pressure)
• High cholesterol levels
• Obesity
• Lack of physical activity
• Diabetes
• Smoking
• Alcohol consumption

If the patient manages to decrease or cut out the risk factors associated with stroke, it is avoidable. Without managing any 2 of the basic 7 modifiable factors, the risk of stroke increases 3 times compared to those who manage all the factors.⁵

Detailed description, coming up ahead, will help you to be aware of the lifestyle changes available for yourself and those close to you, which can help prevent stroke and other complications associated with moyamoya disease.

Diet

Healthy dietary habits can keep the body healthy, and their impact on managing the risk of stroke is certainly remarkable. There is published evidence which further corroborates the fact that the risk of stroke reduces as one practices lifestyle management.

Dietary Approaches to Stop Hypertension (abbreviated as DASH) is a special type of diet routine, which is proven to be useful in the betterment of hypertension and reducing the risk of stroke. 

• Things to include in your diet: Vegetables, fruits, grains (as whole), dairy products (with low fat content), nuts, fish, and protein-rich foods (for example: legumes) are advised to be included in your diet
• Things to avoid in your diet: foods with a high sodium content, meat (red), food items with high sweetening agents

There are numerous advantages of following the diet plan. For example, people with diabetes who follow a dietary routine can better control their blood glucose level. Another benefit is that blood pressure stays at a controlled, healthy level, and bad cholesterol is reduced.

In addition to improving your meal quality, you can also keep track of your calorie intake, which can in weight loss as a way to treat obesity. All these improvements can reduce the risk of stroke.

Physical activity

For patients with moyamoya disease, physical inactivity is one of the main issues, as blood supply to the brain is compromised. Increasing everyday physical activity can significantly improve blood flow and reduce the risk of stroke.

Exercise with medium intensity serves many purposes in improving a person's health, such as decreasing blood pressure, lowering cholesterol levels, helping with weight loss, and increasing insulin activity to improve diabetes. All these lead to a reduced risk of stroke.

It is important to know that patients who have survived a stroke and work to prevent a secondary stroke find it far more difficult to be physically active.

Smoking

Smoking causes many complications, which are related to increasing the risk of stroke, and these include increased heart rate in an extreme manner, blood pressure raises suddenly, and hardened arteries become harder too. In patients suffering from moyamoya disease, all these effects get even worse as brain-related complications are already striking.

These are even more dangerous in hypertensive patients. Moreover, smoking worsens diabetes as well. 4 times, yes, smokers are at 4 fourfold stroke risk when compared with non-smokers.

When smokers quit smoking, all the complications start to get reversed, which means blood pressure and heart rate get normalised; diabetes and lipid profile become better, which reduces the risk of stroke.

Alcohol consumption

Consumption of alcohol acts as a risk factor in raising the blood sugar level and increasing the blood pressure, and both these can act to trigger a stroke. Low consumption of alcohol is associated with reducing the risk of stroke, BUT there is NOT any kind of recommendation from the American Heart and Stroke Association.

Thus, reduced alcohol consumption can certainly improve the process of stroke prevention by controlling blood pressure and diabetes.

All these lifestyle changes help in the management of the existing condition of the patients suffering from moyamoya disease and decrease the chances of further development of any kind of blockage in the brain.^{5, 6}

Barriers in the path of management

Psychological aspects

In a study, patients suffering from moyamoya disease reported that sudden information after the diagnosis of the disease under discussion has put them into confusion and depression.

Anxiety was also reported regarding moyamoya disease, especially the uncertain behaviour of the disease was the major unease. One of the most noticeable concerns that intensely burdened the patients was the fear of disease transfer to their children.

Awareness

It is a vital point to discuss here that if the patient is not aware of the outcomes of the disease, then the patient would not show compliance to get that treated, and in the case of moyamoya, in which patients is already suffering with some serious signs and symptoms might not be in a position to tackle the burden of the information regarding stroke risk. In that situation, the patient might not be willing to do what is required.

Physical barriers

Moyamoya disease patients need to improve the blood flow so that any cluster can be removed from the site where the problem dwells, and other risk factors can be reduced; however, patients with moyamoya face issues related to balancing the body, which makes it hard for them to work out physically.^3,7

How to overcome the barriers

One of the most important boosters to overcome the obstacles is the support from family and friends.

Counselling of the patients in a professional manner can make them compliant.

The environment in which patients live and the people who surround them should also be trained in a way to be with the patient, as confusion, imbalance, and other cognitive issues need special care.^8,9

Patients should be encouraged to participate in the clinical trials, so that further developments can be achieved to find a cure for moyamoya disease.

Since Moyamoya disease is non-curable, accepting it and adapting accordingly is the way to deal with the disease, as patients live with the illness and experience it throughout their life journey.⁷

Summary

Moyamoya is a Japanese word, means puff of smoke or cloud. This term is given to a medical condition, in which blood-carrying vessels in the neck region get narrowed, and when an image of that site is generated in a diagnostic procedure, named angiography, that site looks like moyamoya.

In moyamoya disease, arteries that get narrowed can further get blocked and eventually strokes can take place. Age groups of 10-14 years and 40s are prone to getting attacked, and AFAB are at higher risk as compared to AMAB.

Headache, eyesight issues, imbalanced body, speech problems, weakness, numbness, and lack of concentration are the symptoms associated with moyamoya disease. Surgery of the impacted site can be done to tackle; however, the disease is non-curable. So, symptomatic treatment is done to prevent the risk of stroke.

Lifestyle changes to manage the moyamoya disease are extremely important. Diet, physical activity, Smoking cessation, and quitting alcohol consumption keep the risk factors, like hypertension, high cholesterol, obesity, and diabetes, under control. This reduces the risk of stroke.

Patients suffering from moyamoya disease have reported being confused and depressed after knowing that they had been diagnosed with moyamoya disease. Physical activity is necessary for managing the disease, but the patient might not feel it is easy to perform.

Support from friends and family is really important. Management of the risks can only be done as moyamoya is non-curable. So, accepting it and adapting accordingly is the way of life for the moyamoya patients.