Moyamoya Disease is a rare blood vessel disorder that causes the carotid artery in the skull to become blocked or narrowed. This is a major artery to the brain, and when it becomes blocked, it will cause tiny blood vessels to form at the base of the brain in its place. This condition commonly causes a ministroke, but it can also cause bleeding in the brain. Cognitive and developmental delays can be related to this disease, as well as disability.
This condition most commonly affects children, but it can also be seen in adults. The symptoms differ depending on the age of the person impacted by the disease. In children, the first symptom is often a stroke or a recurrent ischemic attack.
Adults can also experience these symptoms as their first indication of the condition, but adults more commonly have bleeding in the brain as their first symptom of illness. Other symptoms that can be related to Moyamoya disease are:
The exact cause of this condition is not known. The disease is most common in Japan, Korea, and China, but it can be diagnosed in other parts of the world as well. The prevalence of the condition in these specific places suggests that heredity might play a role in the disease. Changes to the blood vessels related to other health conditions, such as Down Syndrome or sickle cell anaemia, are also thought to be a root cause of Moyamoya syndrome.
The risk factors for Moyamoya disease are:
Diagnosis of this health condition requires the help of a neurologist who specialises in moyamoya disease. A full medical history will be taken by this specialist before other tests are run. MRI and CT scans can be used to visualise the veins, arteries, and the brain. A cerebral angiogram might also be done to look at the blood vessels of the brain more closely.
PET scans and EEG can also be used to help identify the condition and behaviour of structures in and around the brain. Surgical transcranial Doppler ultrasounds can be done to obtain images of the head and neck in cases where blood flow problems in the neck are suspected.
The severity of the symptoms that a patient is experiencing can help determine what kinds of interventions will be used to treat Moyamoya disease. The main goal of treatment is to help the patient to avoid having a stroke. Blood thinners are commonly given to patients with this condition to discourage clotting and to ensure that the brain is supplied with blood. Anti-seizure medications are indicated in some cases, and calcium channel blockers might be given to help manage symptoms like headaches.
Surgery might also be the best course of action in severe cases. There are various kinds of surgical options that can be explored, depending upon the recommendation of your doctor. If surgery is not an option, but there is impairment of mental and cognitive function impacting a patient’s well-being, a physical therapist or a speech therapist might be asked to provide supportive care and treatment.
This condition cannot be prevented, and the main course of action that is taken for patients is the prevention of severe secondary side effects and comorbidities.