Description

Moyamoya disease is a rare condition of the blood vessels in the carotid artery in the skull is blocked or narrowed. The carotid artery is a major artery that carries blood to the brain. When it is blocked, the blood flow to the brain is reduced. Small blood vessels that form at the base of the brain and the blood supply to the brain.

The condition can cause a mini stroke, known as a transient ischemic attack (TIA) or a stroke . It can also cause bleeding in the brain. Moyamoya disease can affect how well the brain works and can cause cognitive and developmental delays or disabilities.

Moyamoya disease most commonly affects children. However, adults may have the condition. Moyamoya disease is found all over the world. But it is more common in the countries of East Asia, especially in Korea, Japan and China. This may be due to certain genetic factors in the populations.

Symptoms

Moyamoya disease can occur at any age. But the symptoms are more common in children between the ages of 5 and 10, and in adults between the ages of 30 and 50. Spotting symptoms early is very important to avoid complications such as a stroke.

Moyamoya disease causes different symptoms in adults and children. In children, the first symptom is usually a recurrent stroke or transient ischemic attack, also called TIA. Adults may experience these symptoms as well. But adults can also experience bleeding in the brain, known as a hemorrhagic stroke. The bleeding occurs due to the shape of the blood vessels in the brain formed.

The symptoms of moyamoya disease associated with the reduction of blood flow to the brain include:

  • The pain of a headache.
  • Seizures.
  • Weakness, numbness or paralysis in your face, arm, or leg. This is usually on one side of the body.
  • Vision problems.
  • Difficulty speaking or understanding others, known as aphasia.
  • Cognitive or developmental delays.
  • The involuntary movements.

These symptoms can be caused by exercise, crying, coughing, straining or fever.

Consult your health care professional if you have any of the symptoms of moyamoya disease. The early detection and treatment can help prevent a stroke, and serious complications.

When to see a doctor

Seek immediate medical attention if you notice any of the symptoms of a stroke or mini-stroke, even if the symptoms seem to come and go or disappear.

To verify if there are signs of a stroke, think "FAST" and do the following:

  • Of the face. Ask the person to smile. Does one side of the face drooping?
  • Of the arms. Ask the person to raise both arms. Does one arm drift downward? Or is one arm unable to rise?
  • Speech. Ask the person to repeat a simple phrase. Is the speech slurred or strange?
  • Time. If you notice any of these symptoms, call 911 or emergency medical help immediately.

Do not wait to see if the symptoms disappear. Every minute counts. The longer a stroke goes untreated, the greater the potential for brain damage and disability.

If you're with someone you suspect is having a stroke, watch the person carefully while waiting for emergency assistance.

Causes

The exact cause of moyamoya disease is not known. Moyamoya disease is most commonly seen in Japan, Korea and China. But it also happens in other parts of the world. Because the moyamoya disease is more common in these Asian countries, the researchers believe that this suggests a genetic factor in some populations.

Sometimes, the changes in the blood vessels, known as vascular changes, it may happen that mimic the moyamoya disease. These changes may have different causes and symptoms. This is known as the syndrome of moyamoya.

The syndrome Moyamoya can be linked to certain conditions, such as Down syndrome, sickle cell anemia, neurofibromatosis type 1, and hyperthyroidism.

Risk factors

Although the cause of moyamoya disease is not known, there are certain factors that may increase your risk of having the disease. They include:

  • Asian heritage. Although moyamoya disease is found in all the world, it is more common in the countries of East Asia, especially in Korea, Japan and China. This may be due to certain genetic factors in the populations. People with Asian heritage living in the Western countries also have a higher risk of moyamoya disease.
  • Family history of moyamoya disease. If you have a family member with moyamoya disease, your risk of having the disease is 30 to 40 times greater than that of the general population. This suggests a genetic relationship.
  • Medical conditions. The syndrome Moyamoya sometimes happens with other disorders, such as neurofibromatosis type 1, sickle cell disease and Down syndrome, among many others.
  • The fact of being a woman. Moyamoya disease is slightly more common in women.
  • Be young. Although adults can have moyamoya disease, children under 15 years of age are most commonly affected.

Complications

Most of the complications of moyamoya disease are linked to the effects of the stroke. They include seizures, paralysis, and vision problems. Other complications include problems with speech, movement disorders and delays in development. Moyamoya disease can cause serious and permanent damage in the brain.

Prevention

There is no way to prevent the moyamoya disease. However, moyamoya treatments can prevent strokes and other complications.

Diagnosis

Moyamoya disease is usually diagnosed by a doctor who specializes in conditions of the brain, called a neurologist. The neurologist reviews your symptoms and your medical and family history. The neurologist may also perform a physical exam. Several tests are usually required for a diagnosis of moyamoya disease and any underlying medical condition.

Tests may include:

  • Magnetic resonance imaging.This imaging test is also known as a magnetic resonance imaging. An mri uses powerful magnets and radio waves to create detailed images of the brain. A health care provider may inject a dye into a blood vessel to view the arteries and veins and highlight the circulation of the blood. This type of test is called magnetic resonance angiography. Your neurologist may recommend a perfusion by magnetic resonance imaging. This type of imaging can measure the amount of blood that passes through the blood vessels. It can show if there is a decrease in the blood supply to the brain.
  • Computerized tomography scan. Also known as CT, this imaging test that uses a series of X-rays to create a detailed image of your brain. A health care provider may inject a dye into a blood vessel to highlight blood flow in the arteries and the veins. This is called a computed tomography angiography. This test cannot diagnose early stages of moyamoya disease. But you can help find problems with the blood vessels.
  • Cerebral Angiography. In a cerebral angiogram, a health care professional inserts a long thin tube called a catheter into a blood vessel in the groin. The health professional then uses X-ray imaging to guide the catheter to your brain and is used to inject a dye through the catheter into the blood vessel of your brain. Dye conforms to the shape of the blood vessels to become more visible in the x-ray images.
  • The positron emission tomography scan or single-photon emission computed tomography. In these tests, you are injected with a small amount of a security of radioactive materials. Positron emission tomography, also called PET imaging provides images of brain activity. Single-photon emission computed tomography, called SPECT to the short, measures the flow of blood to the brain.
  • Electroencephalogram. An electroencephalogram is also called an EEG. It controls the electrical activity in the brain using small, metal discs called electrodes are attached to your scalp. Children with moyamoya disease often have EEG results that are outside the typical range.
  • The ultrasound transcranial Doppler. In the ultrasound, transcranial Doppler, sound waves are used to obtain images of your head and sometimes your neck. Specialists can use this test to evaluate the blood flow in the blood vessels in the neck.

Magnetic resonance imaging. This imaging test is also known as a magnetic resonance imaging. An mri uses powerful magnets and radio waves to create detailed images of the brain. A health care provider may inject a dye into a blood vessel to view the arteries and veins and highlight the circulation of the blood. This type of test is called magnetic resonance angiography.

Your neurologist may recommend a perfusion by magnetic resonance imaging. This type of imaging can measure the amount of blood that passes through the blood vessels. It can show if there is a decrease in the blood supply to the brain.

If necessary, your neurologist may order other tests to rule out other conditions.

Treatment

The health professionals to assess your condition and determine the treatment that is right for you. The treatment is not a cure for moyamoya disease. But treatment can help prevent strokes.

The goal of treatment is to reduce symptoms and improve the flow of blood to the brain. The treatment also aims to reduce your risk of complications. Complications include ischemic stroke caused by a lack of flow of blood, bleeding in the brain and death.

The prognosis for moyamoya disease, also called the prognosis depends on several factors, including:

  • How early the disease was diagnosed.
  • How much damage has occurred when seeking treatment.
  • Whether or not to undergo treatment.
  • Age.

Your treatment may include medicines, surgery, and therapy.

Medicine

Medicines may be prescribed to control symptoms, reduce the risk of a stroke or to help in the control of seizures. Medications may include:

  • A blood thinner. Anticoagulants are usually recommended if you have been diagnosed with moyamoya disease and you have mild or no symptoms. Your health care professional may recommend that you take aspirin or other blood thinners to prevent strokes.
  • Calcium channel blockers. Also known as calcium antagonists, this type of medication can help to manage the headaches. It can also help to reduce the symptoms associated with transient ischemic attacks. Calcium channel blockers can help control blood pressure, which can help to prevent the damage of the blood vessels in people with moyamoya disease.
  • Anti-epileptic drugs. These medications may be helpful for those who have had seizures.

Surgery

Early surgical treatment can help to slow the progression of moyamoya disease. Your neurologist may recommend bypass surgery if symptoms develop or stroke. Surgery may also be recommended if the tests show evidence of low blood flow to your brain.

In bypass surgery, the surgeons bypass blocked arteries. They do this by connecting blood vessels in the outer part of the skull to the inside of the skull to help restore the blood flow to your brain. This may include direct or indirect revascularization procedures. Or you may include a combination of both.

  • Direct revascularization procedures.In direct revascularization surgery, the surgeons sew up the scalp of the artery directly into an artery of the brain. This is also known as the superficial temporal artery to middle cerebral artery bypass surgery. This procedure increases the blood flow to your brain immediately. Direct bypass surgery can be difficult to perform in children because of the size of the blood vessels. But it is the preferred choice in adults. This intervention can be performed safely and effectively by an experienced surgical team that treats people with moyamoya disease daily.
  • Indirect revascularization procedures.In indirect revascularization, the goal is to increase the flow of blood to the brain little by little. In adults being treated in high-volume surgical centers, indirect revascularization is almost always combined with direct revascularization. Indirect revascularization procedures include encephaloduroarteriosynangiosis. This is also called EDAS. They include encephalomyosynangiosis, also called EMS. Or include a combination of both. In EDAS, the surgeon makes a small temporary opening in the scalp to expose the artery. Then, the surgeon makes an opening in the skull directly beneath the artery. The surgeon places the intact scalp of the artery on the surface of his brain, which allows the blood vessels of the artery to grow in your brain over time. The surgeon then replaces the bone and closes the opening in the skull. In EMS, the surgeon separates the muscle in the temple region of the forehead and placed over the surface of the brain through an opening in the skull. This helps to restore the blood flow. Your surgeon may perform EMS with EDAS. In this procedure, the surgeon separates the muscle in the temple region of the forehead. The surgeon placed on the surface of his brain after placing the scalp artery to the surface of his brain. The muscle helps to keep the artery instead of the blood vessels grow in your brain over time.

Direct revascularization procedures. In direct revascularization surgery, the surgeons sew up the scalp of the artery directly into an artery of the brain. This is also known as the superficial temporal artery to middle cerebral artery bypass surgery. This procedure increases the blood flow to your brain immediately.

Direct bypass surgery can be difficult to perform in children because of the size of the blood vessels. But it is the preferred choice in adults. This intervention can be performed safely and effectively by an experienced surgical team that treats people with moyamoya disease daily.

Indirect revascularization procedures. In indirect revascularization, the goal is to increase the flow of blood to the brain little by little. In adults being treated in high-volume surgical centers, indirect revascularization is almost always combined with direct revascularization.

Indirect revascularization procedures include encephaloduroarteriosynangiosis. This is also called EDAS. They include encephalomyosynangiosis, also called EMS. Or include a combination of both.

In EDAS, the surgeon makes a small temporary opening in the scalp to expose the artery. Then, the surgeon makes an opening in the skull directly beneath the artery. The surgeon places the intact scalp of the artery on the surface of his brain, which allows the blood vessels of the artery to grow in your brain over time. The surgeon then replaces the bone and closes the opening in the skull.

In EMS, the surgeon separates the muscle in the temple region of the forehead and placed over the surface of the brain through an opening in the skull. This helps to restore the blood flow.

Your surgeon may perform EMS with EDAS. In this procedure, the surgeon separates the muscle in the temple region of the forehead. The surgeon placed on the surface of his brain after placing the scalp artery to the surface of his brain. The muscle helps to keep the artery instead of the blood vessels grow in your brain over time.

Possible risks of the surgery revascularization procedures for moyamoya disease include changes in the pressure in the blood vessels in the brain. This can cause headaches, seizures, and bleeding. However, the benefits of the surgery greatly outweigh the risks.

Some people with moyamoya disease to develop a widening or ballooning of a blood vessel in the brain known as a brain aneurysm. If this happens, surgery may be needed to prevent or treat a ruptured brain aneurysm.

Therapy

To address the physical and mental health of the effects of a stroke and that of your child, your health care professional may recommend an evaluation by a psychiatrist or a therapist. Without surgery, moyamoya disease can cause cognitive impairment due to narrowing of the blood vessels. A psychiatrist can look for signs of problems with thinking and reasoning skills. The psychiatrist can also monitor you or your child for signs of these problems are worsening.

The cognitive-behavioral therapy can help to deal with emotional problems related to the implementation of moyamoya disease, such as how to face the fears and uncertainties about the future of the strokes.

Physical and occupational therapy can help you to recover any loss of physical function caused by a stroke.

Preparing for your appointment

If you or your child has been diagnosed with moyamoya disease, the more likely it is to be referred to a doctor who specializes in the brain of conditions, known as a neurologist.

Here's some information to help you prepare for your appointment.

What you can do

  • Be conscious of any thing you need to do ahead of time. At the time you make the appointment, be sure to ask if there is anything that you or your child needs to do in advance, such as restrict your diet.
  • Write down any symptoms you or your child, including those that do not seem to be related to the reason for which you scheduled the appointment.
  • Write important personal information, including major stresses or recent life changes.
  • Make a list of all medications, vitamins or supplements that you or your child are taking and the dose.
  • Have a family member or friend. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
  • Write questions to ask their health care team.

Your time with your health care team is limited, so preparing a list of questions can help you make the most of their time together. A list of questions from most important to least important in case time runs out. For moyamoya disease, some basic questions to ask include:

  • What is probably the cause of my or my child's symptoms or condition?
  • What tests are needed? Does not require special preparation?
  • What treatments are available, and which do you recommend?
  • What side effects can I expect from the treatment?
  • Surgery is a possibility?
  • Are you prescribing a medication? If so, is there a generic alternative?
  • I have other medical problems. How can they be managed together?
  • I or my child have any restriction of physical activity?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?

What to expect from your doctor

Your healthcare provider is likely to ask a series of questions, such as:

  • When did symptoms first begin?
  • How often do the symptoms occur?
  • Do certain activities trigger the symptoms?
  • Has anyone in your immediate family ever had moyamoya disease?
Symptoms and treatment of Moyamoya disease