Moyamoya is a rare, progressive cerebrovascular disorder brought about by the blockage of arteries at the basal ganglia, or the base of the brain. “Moyamoya” is a Japanese term that means “puff of smoke.” The name describes the appearance of the blood vessels that characterize the disease; the blood vessels are tangled purposely to compensate for the blockage of the arteries. This tangled mess of blood vessels resembles a puff of smoke, for which the disease is so aptly named.
The disease was first introduced in Japan during the 1960s. However, it isn’t confined to one country. Cases of it are also reported fromAustralia, Africa, Europe and theUnited States. The primary group that is affected by Moyamoya is children, although a significant number of cases have been found in adults. The first symptom of the disease is stroke.
Moya Moya disease affects the blood supply to the brain. Symptoms such as these resemble with the symptoms of a stroke. This is still a rare disorder, although now it is more frequently successfully diagnosed because of the recent technologies in diagnostic imaging. Moyamoya occurs most often on children with ages 5-15 and also during adulthood between ages 30-40.
Once the process of blockage or vascular occlusion occurs, it cannot be stopped by any medical management unless surgery takes place. Repeated strokes to people with MMD can have severe functional impairment and in severe cases, even death. Thus, it is really important to be managed and treated on time. Without proper and timely treatment, the disease will cause progressive neurological deterioration and massive internal bleeding, which can lead to shock and death.
In children, the most common presenting symptoms of MMD are stroke of transient ischemic attacks (TIAs). These neurological symptoms are often coupled with paralysis of the body, seizures and severe muscular weakness that can be extremely debilitating. Also, in both children and adults, the most common symptom is stroke. Other symptoms that appear in both children and adults are altered consciousness, aphasia or speech difficulties, vision problems, difficulty with cognitive and sensory functions and involuntary movements. The majority of patients with Moyamoya will also suffer from progressive cognitive deterioration, which can eventually lead to intracerebral hemorrhage and death.
Today, there is still no known direct cure for Moyamoya disease, but there are treatments that can help manage the signs and symptoms that are associated with the disease.
As mentioned before, this disease can lead to several episodes of weakness, paralysis in a limb or of one side of the body (hemiparesis), headaches, dizziness and seizures. There is also a risk of mental retardation, as well as sustained neurological problems. The occurrence of TIA or transient ischemic attack is one of the severe possible immediate problems that can be experienced with Moyamoya disease. During TIA, the blood flow towards the brain is blocked completely, but only temporarily. Other immediate problems that should be addressed are stroke, which is a sustained complete blockage of the blood flow to the brain and finally there are the risk of having intracranial hemorrhage or bleeding in the brain. Immediate treatments should be done for these problems in order to keep these symptoms controlled and to keep the symptoms from progressing to potentially fatal symptoms.