What is Moya Moya Syndrome؟
Moya Moya syndrome is a progressive disorder that affects the blood vessels of the brain (cerebrovascular). When the main artery that supplies blood to the brain, the small blood vessels at the base of the brain open at the end to supply blood to the brain. These small veins are the moyamoya veins for which the disease is named. Inadequate blood supply leads to a decrease in oxygen supply to the brain, and it is this lack of oxygen that causes the symptoms of moyamoya.
One of the symptoms of Moya Moya syndrome is a stroke. That leads to paralysis of the face, arms, legs, loss of speech, or temporary loss of nerve function of body parts or speech. Other symptoms that occur include headaches, visual disturbances, developmental delays, and seizures. Approximately 10 percent of moyamoya cases in Asian countries are genetically caused. It is said that patients in whom arterial changes occur along with another process, such as sickle cell disease or Down syndrome. They have Moya Moya Syndrome.
Symptoms of Moya Moya Syndrome
The symptoms of moyamoya disease are variable and depend on the age of the patient. They occur in children between the ages of 5 and 10 and in adults between the ages of 30 and 50. The first symptom of moyamoya disease is usually a stroke or frequent transient (mild) ischemic strokes, especially in children.
Headaches
Seizure
Weakness, numbness, or paralysis in the face, hands, or legs, usually occurring on one side.
Visual impairments
Problems speaking or understanding what others are saying (aphasis)
Delay or slowness in growth
Involuntary movements
Decreased power of perception
Exercising, crying, coughing, straining or fever are some of the things that can trigger these symptoms.
Diagnosis of Moya Moya Syndrome
MRI
CT Scan
Cerebral angiography CT
Doppler ultrasound of the cerebral arteries
PET (Positron Emission Tomography) or SPECT (Single Photon Emission Computed Tomography)
Progression of Moya Moya Syndrome
People who develop this condition go through different stages. Initially, the carotid arteries constrict and provide the conditions for the progression of the disease. Next, the blood vessels, known as moya moya veins, begin to grow at the basal part of the brain.
The faster the growth of the vessels, the narrower the thickness of the carotid artery vessels and the fewer of them. In the last stage of the progression of the disease, the moya moya arteries are completely destroyed and the carotid artery vessels are blocked. As these vessels become blocked, the secondary blood vessels in the skull will also increase.
Treatment of Moya Moya Syndrome
Treatment measures include taking aspirin to prevent or reduce the development of small blood clots that form in narrow veins. Use of anticonvulsant medications if the patient has seizure disorders. In rare cases, anticoagulant drugs such as Lovenox or Coumadin are prescribed in patients with a very acute condition with recurrent symptoms. But because of the risk of brain bleeding in these conditions, they are rarely prescribed as long-term measures. Calcium channel blockers are sometimes used to help reduce headaches and, in some patients, to reduce symptoms related to transient ischemic attacks. However, calcium channel blockers must be used carefully. Because they can lower blood pressure, which increases the risk of stroke. There is no medication available that stops the progression of cerebral artery narrowing.
Many surgical methods have been suggested to treat moyamoya. And they are divided into so-called indirect and direct actions. The indirect procedure is usually performed in children and younger patients.
This surgery includes pyal synangiosis, encephalomyosinangiosis (EMS), encephalodorartriosinangiosis (EDAS), dural inversion, and other similar types.