Moyamoya Disease

Moyamoya disease is a rare condition where the arteries supplying oxygen and essential nutrients to the brain gradually become narrow. This causes a network of small blood vessels to bypass the blockage. The condition can progress and result in medical emergencies such as brain haemorrhage, transient ischemic attack (numbness, weakness, speech impairment or visual abnormalities) and paediatric stroke.

Symptoms

Moyamoya disease causes stroke-like symptoms such as weakening on one side of the body, blurred vision, slurred speech, headaches and sometimes seizures. Brain tissue damage can result in headaches, cognitive or learning difficulties, seizures and mental impairment.

Diagnosis and Treatment Options

To diagnose Moyamoya disease in your child a physical and neurological examination of your child is carried out to identify signs and symptoms and family medical history is taken. Diagnostic tests such as x-rays, computed tomography (CT) or magnetic resonance imaging (MRI) scans are carried out.

Sometimes a cerebral angiogram is needed to confirm diagnosis. This is a test that shows the blood vessels in the brain with the help of a dye. It involves inserting a long catheter into a groin artery and threading it to the brain using x-ray imaging to show the blood vessel network. Other imaging tests may be needed to identify areas of the brain with diminished oxygen supply.

Surgery is usually needed to prevent stroke and permanent brain impairment. This involves redirecting blood towards the oxygen-deficient areas of the brain from other parts of the head such as arteries in the scalp or muscles. In the meantime, some medications can help to manage your child’s symptoms. These can include anti-clotting drugs such as aspirin and calcium channel blockers to stop calcium from entering the heart cells and blood vessel walls. This helps to lower blood pressure and reduce headaches.

Recovery

• Some confusion and possible neurological deficits
• Rehabilitation
• Radiation/ chemo therapy
• Follow up MRI
• Possible follow up surgery
• Physiotherapy
• Occupational therapy
• Psychological support if needed
• Pain medication as required
• Speech therapy