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Discovering the Moyamoya Disease

8th November 2007

Moyamoya means “a puff of smoke” in the Japanese language. This is how the Japanese had described how the tiny vessels look when they become blocked, causing blood not to freely flow through. The disease is discovered in Japan by the 1960′s but it was also seen in people living in Europe, Africa, Australia, and the United States.

However, the Moyamoya disease is very rare affecting only one in two million people in the U.S. This disease involves the brain as well as the heart. In this disease, the arteries found at the base of the brain, more commonly called the basal ganglia, are blocked.

Symptoms of the Moyamoya Disease
Medical experts are zeroing in on the theory that the Moyamoya disease is a hereditary disease. Most of its cases run within families. With this, researchers are concluding that the disease could have resulted from an inherited genetic disorder.

The Moyamoya disease primarily targets the children although a number of adult cases are recorded as well. The main symptom of this disease is stroke or mild ischemic attacks. Patients are also going to experience general weakness of the body, which may eventually lead to partial paralysis and seizures.

And of the children affected with the Moyamoya disease, it is observed that the instances are more frequent in women than it is in men. And as these female children grow up, they may experience complications upon reaching the childbearing age. They may experience several problems associated to both pregnancy and the delivery of the baby, although the findings related to this issue are not yet conclusive.

In adults, instances of hemorrhagic stroke are also documented. Adult patients also suffer from speech deficits and they also tend to drift into unconsciousness from time to time. Their cognitive and sensory capabilities also become impaired and that includes their vision.

Diagnosis of the Moyamoya disease
To accurately detect the Moyamoya disease, doctors usually request for a CT scan, or a computerized tomography of the head, more particularly on the part where the blood vessel is blocked. Aside from a CT scan, they may also need a magnetic resonance image of the affected are as provided by the MRI. An angiography, or an x-ray of the blood vessels involved is needed as well.

A newer diagnosis tool called the SPECT, or more particularly the single photon emission computerized tomography, is now performed. This deviced is used to effectively display the effects of the deceased blood and oxygen supply to the affected areas of the brain.

Treatment of the Moyamoya disease
The drugs commonly used to treat this disease are the blood thinning medicines referred to as the warfarin. Corticosteriod medications are also given. However, the primary treatment of the Moyamoya disease is the revascularization surgery. This is a type of surgery that repairs the narrowed opening of the blood vessels so as to restore the flow of blood to the brain.

Discovering the Moyamoya Disease But to perform this surgery, an extracranial-intrcranial bypass has to be done first. This procedure also ensures that the instances of strokes related to the disease are reduced. This surgery is more effective in children, but a lot of adult patients who undergo it have lesser to zero occurrence of stroke after surgery.

In milder cases, patients may not need surgery but just a draining of the cerebrospinal fluid from the ventricular system. However, this procedure is performed on adult patients only.

In highly rare cases, organ transplantation is carried out. But this is not frequently done because it is not only critical; it also has a lot of medical and legal implications. Fatal cerebral hemorrhage may happen if the procedure is not done and monitored correctly.

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