Moyamoya disease is a rare, progressive blood vessel (vascular) disease in which the carotid artery in the skull is blocked or narrowed and blood flow to the brain is reduced. The tiny blood vessels are then opened to the brain at the base of the brain to provide blood. Gelir Moyamoya olan means cigarette smoke, a term that defines the appearance of this small set of blood vessels in Japanese.

These small blood vessel clusters cannot provide the brain with the necessary blood and oxygen, resulting in temporary or permanent brain injury. This may cause a ministroke (transient ischemic attack or TIA) in a blood vessel (aneurysm), paralysis, swelling, swelling or bleeding in the brain. It can also affect the performance of your brain, causing cognitive and developmental delays or disability. Moyamoya disease affects children most frequently, but can also be seen in adults. Moyamoya disease is found all over the world, but it is more common in East Asian countries, especially in people from Korea, Japan, China and East Asia.

symptoms

Moyamoya disease may occur at any age, but the most common symptoms are between 5 and 10 years of age in children and 30 to 50 years of age in adults. The first symptom of Moyamoya disease is stroke or recurrent transient ischemic attacks, especially in children (TIA). Adults may also experience these symptoms, but more often the brain receives hemorrhagic stroke from the abnormal brain vessels. Symptoms and symptoms associated with moyamoya disease associated with reduced blood flow to the brain are as follows:

• Headache

• Seizures

• In the face, arm or leg, usually weakness, numbness or paralysis on one side of the body

• Visual disturbances

• Difficulties in speaking or understanding others (aphasia )

• Developmental delays

• Involuntary movements

• Cognitive decline

These symptoms can be triggered by exercise, crying, coughing, strain, or fever.

When to see a doctor?

If any stroke or TIA symptom or symptom is noticed, if it dwindles or disappears, immediately seek medical attention.

Diagnosis

Doctors use the latest tools and techniques to diagnose moyamoya disease. Moyamoya reviews the symptoms of the doctor, family and medical history to diagnose the disease. By performing a physical examination, the doctor may request a number of tests to diagnose moyamoya disease and the underlying conditions. The tests required are;

• Magnetic resonance imaging (MRI):An MRI uses powerful magnets and radio waves to create detailed images of the brain. The doctor can inject the dye into a blood vessel (magnetic resonance angiography) to view the veins and emphasize blood circulation. If appropriate, the doctor may recommend an imaging (perfusion MRI) that can measure the amount of blood passing through the vessels.

• Computed tomography (CT) scan: A CT scan uses a series of X-rays to create a detailed image of the brain. The doctor may inject a dye into a blood vessel to emphasize the blood flow in the vessels (CT angiogram).

• Cerebral angiography:During a brain angiogram, the doctor adds a long thin tube (catheter) to the blood vessel in the groin and directs it to the brain using X-ray imaging. Then, the X-ray injects the dye into the blood vessels of the brain through the catheter to allow them to appear under imaging.

• Transcranial Doppler ultrasound: In a transcranial Doppler ultrasound, sound waves are used to obtain images of the brain. They can use this test to obtain information about blood vessels in the brain.

• Positron emission tomography (PET) or single-photon emission computed tomography (SPECT):In these tests, the physician injects a small amount of safe radioactive material into the person and places the emission detectors on the brain. Pet provides visual images of brain activity. Spect measures blood flow to various parts of the brain.

• Electroencephalography (EEG) electrical activity in the brain EEG traces A through a set of electrodes attached to the head skinless. Children with Moyamoya disease usually show abnormalities in EEG.

If necessary, the doctor may ask for further tests to rule out other conditions.

Treatment of Moyamoya Disease

Doctors; moyamoya patients offer comprehensive treatment. Evaluate the situation and determine the most appropriate treatment. The aim of the treatment is to reduce the risk of serious complications such as reducing symptoms, improving blood flow and ischemic stroke caused by lack of blood flow such as bleeding in the brain (intracerebral hemorrhage) or death.

Medicine

To reduce the risk of stroke or to assist with seizure control, the following drugs can be given:

• Blood thinners: After the diagnosis of Moyamoya disease, it may suggest aspirin or other blood thinner to prevent paralysis if there is mild or no symptoms at first.

• Calcium channel blockers: This type of medication, also known as calcium antagonists, can improve headache symptoms and reduce symptoms of transient ischemic attacks.

• Anti-seizure medications: These can be prescribed if the person has a concomitant seizure disorder.

Revascularization Surgery

The direct revascularization procedure for Moyamoya disease is as follows: If the

symptoms deteriorate or if the tests show evidence of low blood flow, they may recommend revascularization surgery. In revascularization surgery, surgeons bypass clogged arteries to restore blood flow to the brain. The doctor may use either direct or indirect revascularization procedures or a combination of both.

Direct revascularization procedures: In direct revascularization surgery, surgeons directly suture the scalp directly to the brain artery (the superficial temporal artery to the middle cerebral artery bypass surgery) to increase blood flow to the brain. Direct bypass surgery in children, due to the size of blood vessels to be installed may be difficult to do. Direct revascularization surgery has a risk of complications including stroke.

Other Surgical Methods

Some people with moyamoya disease develop a blood vessel swelling or swelling in the brain known as brain aneurysm. If this occurs, surgery may be necessary to prevent or treat a ruptured brain aneurysm.

Therapy

In order to address the physical and psychological effects of stroke on a person, the doctor may recommend other treatments. Physical and occupational therapy can help attempt to regain lost physical functions from stroke. Speech therapy can also be recommended if necessary. Cognitive behavioral therapy can help address emotional problems associated with moyamoya disease, such as how to deal with fear and uncertainty about future stroke. If the person has been diagnosed with moyamoya disease, he is probably referred to a doctor who is a specialist in brain conditions (neurologist).

What a person should do

• If symptoms occur, symptoms should be written somewhere, and then a doctor’s appointment should be adjusted immediately.

• A list of all drugs should be made and details, including dosages, should be indicated. In addition, vitamins and supplements should be included in this list.

• Ask a family member to come to the doctor with you, because sometimes it may be difficult to remember all the information given during an appointment. Someone who comes with you can remember something you missed or forgotten.

• Make a list of the questions you want to ask the doctor and be prepared for a doctor’s appointment.

Some basic questions to ask for are as follows:

• What causes symptoms or condition of my disease?

• What kind of tests are required? Does it require special preparation?

• Which treatments are available and which ones do you recommend?

• What kind of side effects can be expected from the treatment?

• Is surgery possible?

• Is medication administered? Or is there another general alternative?

• Do I have other medical problems? How can they be managed together?

• Is there any restriction on my physical activity?

• Is there any brochure or printed material that I can use? Which websites do you recommend?

What are the expected questions of the doctor?

The doctor is likely to ask a series of questions to the patient. These questions are as follows:

• When did the symptoms first appear?

• How often are the symptoms seen?

• Do some activities trigger symptoms?

• Have any of your family had moyamoya disease?

It is possible to quickly check the signs of the disease by performing the following practical control:

• Face: Asking for a smile, hanging on one side of the face? he checked.

• Balance: The person is asked to remove both arms. Is an arm sliding down? Or he can’t raise an arm up? he checked.

• Speaking:The person is asked to repeat a simple sentence. Is his speech blurry or strange? he checked.

If any of these symptoms are seen, urgent medical attention should be obtained. Certainly not to wait for the symptoms to pass, every minute is important. The longer the stroke is treated, the greater the potential for brain damage and disability. If you are near someone who is suspected of having a stroke, the person should be watched carefully while waiting for help.

Causes of Moyamoya Disease

The exact cause of Moyamoya disease is unknown. Moyamoya disease is more common in Japan, Korea and China, but is also seen in other parts of the world. Researchers have insisted that higher concentrations of moyamoya disease in these Asian countries may have genetic causes for the disease. Moyamoya is also associated with certain conditions such as Down’s syndrome, sickle cell anemia, neurofibromatosis type 1 and hyperthyroidism.

Risk factors

Although the cause of Moyamoya disease is unknown, some factors may increase the risk of a situation involving:

• Asian origin: Moyamoya disease is found worldwide, but is more common in East Asian countries, especially in Korea, Japan and China. This may be due to certain genetic factors in these populations. The higher proportion of Asians living in Western countries has been documented as having this disease.

• Family history of moyamoya disease: If there is a family member with Moyamoya disease, the risk of developing this disease is 30 to 40 times higher than the general population. It is a factor that proposes a genetic component to the disease and justifies the screening of family members.

•To have a certain medical condition: Moyamoya disease neurofibromatosis type 1 sometimes occurs with other disorders such as sickle cell anemia and Down syndrome.

• Being a woman: Women have a slightly higher moyamoya disease.

• Being young: Children under the age of 15 are most affected, although adults have moyamoya disease.

complications

Most complications due to Moyamoya disease are associated with the effects of stroke, such as:

• Vision problems. As a result of stroke, some people with moyamoya disease experience visual disturbances.

• Weakness (hemiparesis)

• Language disorder (aphasia)

• Movement disorders: In some cases, some people with moyamoya disease experience involuntary movements of some muscles.

• Learning or developmental problems: After a stroke, the child may have problems with mental functioning, which may affect school life and also cause emotional difficulties and low self-confidence. Adults may experience a reduction in memory as well as difficulties in other areas of cognitive functioning.

• Seizures

Persons who have noticed at least one of the symptoms of Moyamoya disease should receive immediate medical care without waiting for the indicated relieving or loss. Otherwise the damage to the dealer and the potential for disability will increase.

References:

mayfieldclinic.com

ninds.nih.gov