According to the expert, strokes or recurring headaches are usually the initial signs of moyamoya disease.
moyamoya disease is a rare and progressive cerebrovascular disease caused by blockage of the arteries in the basal ganglia at the base of the brain. In Japanese, moyamoya means ‘puff of smoke’ and refers to the tangled appearance of small capillaries that compensate for the obstruction.
Dr Pavan Pai, Consultant Interventional Neurologist and Stroke Specialist at Wockhardt Hospitals Mira Road says: “The precise etiology of moyamoya disease is uncertain. However, there appear to be both inherited and acquired variants. Researchers are currently looking at genes that could be passed from parent to child.”
Moyamoya disease is sometimes associated with other disorders and is called the moyamoya syndrome or phenomenon. Other conditions to consider include:
- Down’s Syndrome.
- Graves’ disease is a genetic disorder.
- Neurofibromatosis type 1 is a genetic disorder.
- Sickle cell disease is a genetic disorder.
- Atherosclerosis.
- Radiation vasculopathy.
Symptoms
Stroke or recurrent transient ischemic events are often the initial signs of moyamoya disease (TIA). These are known as ‘mini strokes’. Other signs and symptoms may include:
- Hemorrhage in the brain (bleeding).
- Headaches
- slow development
- Aneurysm (bulge or bulge of a blood vessel, which can burst) (bulge or bulge of a blood vessel, which can burst).
- Unwanted movements (when parts of your body move without your control).
- Problems with cognitive skills (such as learning, remembering, and paying attention).
- Problems with the senses (sight, hearing, smell, touch, taste).
- Seizures.
Diagnosis
If your doctor thinks you have moyamoya disease, you may need the following tests:
Cerebral angiography: Your doctor inserts a small tube called a catheter into an artery in your arm or leg. They then inject an iodine contrast dye into your circulation using it. The dye in your blood vessels is then scanned with X-rays. This procedure can map blood flow patterns and determine how much the blood arteries have constricted.
Magnetic Resonance Angiography (MRA): In this painless test, your provider examines your blood arteries for any unique features using a magnetic field, radio waves, and a computer.
Magnetic Resonance Imaging (MRI): MRI uses comparable technology to capture pictures inside your body and determine how your blood is flowing.
Treatment
Moyamoya disease therapy includes symptom control, increased blood supply to the brain, and seizure control. Blood thinners can help prevent clots and blockages, but they can also increase the risk of bleeding. Before suggesting any medication, the doctor will thoroughly analyze the individual condition of each patient.
Revascularization may be beneficial in some circumstances. This is a complex technique performed by a skilled vascular neurosurgeon to restore blood supply to the lower part of the brain. To avoid a stroke in the postoperative period, pain control and careful monitoring of the patient is necessary.
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