About moyamoya syndrome

What is moyamoya syndrome?

Moyamoya syndrome is a progressive disorder that affects the blood vessels in the brain (cerebrovascular). It is characterized by the narrowing (stenosis) and/or closing (occlusion) of the carotid artery inside the skull, the major artery that delivers blood to the brain. At the same time, tiny blood vessels at the base of the brain open up in an apparent attempt to supply blood to the brain distal to the blockage. These tiny vessels are the "moyamoya" vessels for which the disease was named. Inadequate blood supply then leads to reduced oxygen delivery to the brain, and it is this oxygen deprivation that causes the signs of moyamoya. One of the symptoms is typically stroke, which results in paralysis of the face, arms or legs, loss of speech, etc., or temporary loss of neurologic function of body parts or speech (transient ischemic attacks). Other symptoms that may result include headaches, visual disturbances, developmental delay, and seizures. Approximately 10% of cases of moyamoya in Asian countries have a genetic cause. Patients with this arteriopathy that occurs either on a familial or idiopathic basis are said to have moyamoya disease. Patients in whom the artery changes occur in association with another process such as sickle cell disease or Down syndrome are said to have moyamoya syndrome. In this report, we use the term "moyamoya syndrome" as a shorthand for both forms.

What are the symptoms for moyamoya syndrome?

Difficulties with speaking or understanding others (aphasia) symptom was found in the moyamoya syndrome condition

Moyamoya syndrome is defined as a progressive condition that disturbs the blood vessels in the brain. One of its earliest signs of evidence is stenosis or occlusion of the carotid artery present in the skull. Simultaneously, this blockage of blood impels the minute blood vessels at the base of the brain open up. It is said to be an attempt to improve the blood supply. The tiny blood vessels --named moyamoya-- give the disease its name.

Symptoms of Moyamoya Disease
The symptoms of Moyamoya syndrome in children show between the age group of 5 to 10 years, and in that of adults, they may occur between 30 to 50 years old. It causes varying symptoms in children and adults.

Symptoms specific to children:
1. Stroke
2. Recurrent Transient Ischemic Attack

Symptoms specific to adults:
Hemorrhagic Stroke

Other general symptoms triggered through exercising, crying, coughing, straining and Fever are as follows:
1. Headache
2. Seizures
3. Weakness in the arms, legs and face
4. Paralysis of arms, legs or face, typically in just one side of the body
5. Visual problems
6. Issues in speech
7. Difficulty in cognition (aphasia)
8. Cognitive delay
9. Growth delays
10. Involuntary movements

Symptoms
Headache,Seizures,Weakness, Numbness or Paralysis in your face, arm or leg, typically on one side of your body,Visual disturbances,Difficulties with speaking or understanding others (aphasia),Cognitive or developmental delays,Involuntary movements
Conditions
Mini Stroke (transient ischemic attack),Stroke,Bleeding in the brain
Drugs
Blood thinners,Calcium channel blockers,Anti-seizure medications

What are the causes for moyamoya syndrome?

Moyamoya syndrome is classified as a rare blood vessel condition. Here, the carotid artery in the skull is blocked or narrowed, creating a significant gap in the blood flow to the brain. As a cover-up measure, tiny blood vessels, called the Moyamoya, develop at the base of your brain.

Causes of Moyamoya Syndrome
Research says that the Moyamoya Syndrome is mostly seen in Japan, Korea, China, and other parts of Asia. This study suggests that it could be genetically inherited. However, the pinpoint cause is unidentified.

1. Hereditary
2. Vascular changes
It could be associated with other syndromes such as:
1. Down Syndrome
2. Sickle cell anemia
3. Neurofibromatosis type-1
4. Hyperthyroidism
5. Defect in the gene RNF213

Symptoms
Headache,Seizures,Weakness, numbness or paralysis in your face, arm or leg, typically on one side of your body,Visual disturbances,Difficulties with speaking or understanding others (aphasia),Cognitive or developmental delays,Involuntary movements
Conditions
Mini Stroke (transient ischemic attack),Stroke,Bleeding in the brain
Drugs
Blood thinners,Calcium channel blockers,Anti-seizure medications

What are the treatments for moyamoya syndrome?

Moyamoya syndrome is a rare genetic disorder concerned with the blood vessels (vascular) in the brain. Because the carotid artery in the brain is blocked, experiencing blood distal, smaller brain vessels at the base of the brain open up to improve the supply of blood to the brain. These tiny blood vessels are termed moyamoya. Hence, the disease gets its name.

Treatments for Moyamoya Syndrome
Very few treatments completely cure a disease, so such expectations can be unrealistic. There is a wide range of treatments available at the patient’s disposal to effectively prevent strokes. The main objective of treatments is:
1. To lessen the symptoms
2. Enhance the blood flow
3. Reduce the risks of severe complications like ischemic stroke and death

Treatments include the following:
1. Direct Revascularization Procedure: Surgeons stitch your scalp artery directly to the artery in the brain in order to immediately improve the blood flow to the brain.

2. Indirect Revascularization Procedure: The aim here is to lay over the brain's surface blood-rich tissues to gradually increase the flow of blood to the brain over time. High-volume surgical centers almost always combine the procedures of both surgeries in adult patients.

The types of Indirect Revascularization are listed below:
1. EMS: The surgeon may dissect a muscle in the temple region of your head. He would then place it upon your brain surface via an opening in your skull.

2. EDAS: The surgeon separates a scalp artery over numerous inches.

3. EMS and EDAS: Both procedures are combined. The muscle dissected from the temple area of your forehead is after the scalp artery is attached to the surface of the brain, then also placed on the surface of the brain. This muscle aids in holding the artery in place as the blood vessels gradually grow into the brain.

Symptoms
Headache,Seizures,Weakness, numbness or paralysis in your face, arm or leg, typically on one side of your body,Visual disturbances,Difficulties with speaking or understanding others (aphasia),Cognitive or developmental delays,Involuntary movements
Conditions
Mini Stroke (transient ischemic attack),Stroke,Bleeding in the brain
Drugs
Blood thinners,Calcium channel blockers,Anti-seizure medications

What are the risk factors for moyamoya syndrome?

Moyamoya syndrome is a progressive condition in which the blood vessels in the brain are slowly deteriorating. This puts the brain at risk of low blood flow. In an attempt to resolve this, small blood vessels at the base of the brain open up to improve the supply of blood to the brain. This disease gets its name from the little blood vessels called Moyamoya.

The Risk Factors of Moyamoya Syndrome
While the root cause of this disease is unidentified, some factors may have an active role in increasing your risk of developing it.

1. Asian Heritage: It is seen over and over again that Moyamoya Syndrome is concentrated in East and West Asia.

2. Familial Disease: Having a family member with this disease will shoot your chance of inheriting it by 30-40 times.

3. Being female: Higher risk of developing this syndrome.

4. Youth: Although adults are the primary patients, children below the age of 15 are very much vulnerable to Moyamoya syndrome.

Symptoms
Headache,Seizures,Weakness, numbness or paralysis in your face, arm or leg, typically on one side of your body,Visual disturbances,Difficulties with speaking or understanding others (aphasia),Cognitive or developmental delays,Involuntary movements
Conditions
Mini Stroke (transient ischemic attack),Stroke,Bleeding in the brain
Drugs
Blood thinners,Calcium channel blockers,Anti-seizure medications

Is there a cure/medications for moyamoya syndrome?

It is very unlikely for the patient’s condition to improve without resorting to treatments and revascularization surgery. Moyamoya disease can be fatal due to internal bleeding in the brain (intracerebral hemorrhage). Because it is a progressive condition, affected individuals can suffer from cognitive and neurological decline from ischemic stroke or hemorrhage.

Cure/medications for Moyamoya Syndrome:
Although there is no known cure for Moyamoya syndrome, prescribed medications to help manage symptoms and make seizures bearable. Also, it reduces the risk of a stroke.

1. Blood thinners: For mild to no symptoms after diagnosis, blood thinners like aspirin are recommended to prevent strokes.

2. Calcium channel blockers: For headaches and blood pressure control, these calcium antagonist drugs are prescribed. It prevents excessive blood vessel damage and declines symptoms of transient ischemic attacks.

3. Anti-seizure drugs: Medications for those experiencing seizures as a symptom.

Symptoms
Headache,Seizures,Weakness, numbness or paralysis in your face, arm or leg, typically on one side of your body,Visual disturbances,Difficulties with speaking or understanding others (aphasia),Cognitive or developmental delays,Involuntary movements
Conditions
Mini Stroke (transient ischemic attack),Stroke,Bleeding in the brain
Drugs
Blood thinners,Calcium channel blockers,Anti-seizure medications

Video related to moyamoya syndrome