Moyamoya disease

The article was written by Doctor Mai Xuan Thien - Emergency Department - Vinmec Times City International General Hospital.

Moyamoya disease most commonly occurs in children, but can also occur in adults, is detected worldwide, but is more common in East Asian countries, especially Korea, Japan and China, possibly due to genetic factors in these populations.

1. Overview

Moyamoya is a rare cerebrovascular disease, when the intracranial carotid artery is narrowed or blocked, reducing cerebral perfusion, and small blood vessels proliferate at the base of the skull to compensate.
The disease can cause stroke-like symptoms or transient cerebral stroke. The disease can affect brain function and cause cognitive impairment or brain development, and can even cause disability.

2. Symptoms

Moyamoya disease can occur at any age, however symptoms usually appear between the ages of 5-10 years in children and 30-50 years in adults.
The first symptom of the disease is often such as ischemic stroke or recurrent transient ischemic stroke, especially in young children. Adults can develop these symptoms but are usually caused by bleeding in the brain from abnormal blood vessels.
Signs and symptoms of moyamoya disease usually due to decreased cerebral blood flow include:
Headache Convulsions Weakness, numbness, or paralysis of the face, arms, or legs typically on only one side Visual disturbance Loss of speech (Motion disorder) language, or ability to understand language) Developmental delay Disorders of automatic movements Symptoms may have triggers such as exercise, crying, coughing, or fever.

Đau đầu nhức mắt — Bệnh Moyamoya có thể gây ra triệu chứng đau đầu và rối loạn thị giác cho người bệnh

3. Cause

The exact cause of the disease is unknown. The disease is common in Japan, Korea, and China but can also occur in other parts of the world. Researchers believe that the disease is linked to genes in certain populations.
Moyamoya is also associated with certain diseases such as Down syndrome, anemia caused by erythrocytosis, hyperthyroidism,...

4. Risk factors

Although the cause of the disease is unknown, factors that may increase the risk are known as:
Asians: The disease is found worldwide but is common in East Asian countries especially Korea, Japan and China. Family members who have been diagnosed with Moyamoya: If there is a member of your family with Moyamoya disease, your risk is 30-40 times higher than the general population. Having some underlying diseases related to Moyamoya disease: Down syndrome, hyperthyroidism, anemia due to erythrocytosis, ... Female: Females have a higher rate of disease than males Young age: The disease is common. onset in children under 15 years of age.

5. Complications

Complications of the disease related to the consequences of a stroke, for example:
Visual disturbances Movement disorders Impact on learning and development: After a stroke, children may have cognitive development problems , Thinking. May affect the acquisition of knowledge as well as have a negative impact on emotions, causing mild autism.

Rối loạn vận động — Đột quỵ có thể gây hậu quả như rối loạn vận động ở người bệnh

6. Diagnosis

To diagnose the disease, it is necessary to rely on signs, symptoms, personal and family history. Physical examination may reveal some signs of stroke but are not specific. Diagnosis is mainly based on imaging findings.
Cranial magnetic resonance with drugs Computerized tomography brain angiography Transcranial Doppler ultrasound PET or SPECT EEG Vinmec Times City Hospital uses SPECT/CT Discovery NM/CT 670 Pro system with the most modern 16-sequence CT of Vietnam GE Heathcare (USA), the world's leading medical equipment company, provides high quality images, helping to diagnose diseases that need to be investigated early. With a civilized, polite, safe and sterile medical examination and treatment space. Customers when choosing to perform diagnostic tests here can be completely assured of the accuracy of the results.

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7. Treatment

Depending on the condition and stage of the disease, there are different treatment plans.
Treatment purposes: Improve symptoms, increase cerebral perfusion, reduce the risk of dangerous complications such as cerebral ischemia, cerebral hemorrhage or death.
7.1 Drug therapy Anticoagulants or antiplatelets Calcium channel blockers Anticonvulsants 7.2 Reperfusion surgery If symptoms are severe or tests show poor cerebral perfusion, revascularization surgery should be performed. Perfusion may be an option. For example, bypass surgery to restore cerebral perfusion using indirect techniques, direct techniques, or both.

8. Therapy

Depending on the extent or complications of the disease, it may be necessary to undergo different therapies to restore function, cognition, movement, language,...

Reference source: Mayoclinic.org

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