A Puff Of Smoke....

March 11th, 2011

Gotcha! It is not even close to what you were guessing this article was going to be on!

Actually, this article is in response to a patient of mine who asked me about this particular disease and wanted to know more for her own information. So I figured...the information would be great for everyone.

"A puff of smoke"....the meaning of a Japanese word that names a rather rare cerebrovascular condition...Moyamoya Disease. It is caused by the blockage of arteries (from both constriction or clots) at the base of the brain in affected individuals. The descriptive name is from the appearance of the tiny blood vessels that form in the body's attempt to bypass the blockage. The little tangle of new blood vessels resembles a puff of smoke on imaging.

The disease can affect adults (usually in the 30's to 40's), but it is primarily found in children. It often shows itself as unusual strokes or mini-strokes in an age group you would not expect them--children. The stroke symptoms can be accompanied by weakness and sensory loss on the affected side. Sometimes even seizures can result. Other findings that are associated with Moyamoya disease are speech difficulties, a decreased level of consciousness, vision problems, involuntary movements, disturbances in the ability to mentally function, migraine-like headaches, and possibly some other sensory deficits.

Interestingly, the condition often runs in families and is strongly believed to be an inherited disease. Current research has some evidence that the condition is linked to a particular position on a particular chromosome of the human DNA chain. In addition to its inherited potential, the disease can also be acquired. The most common conditions where Moyamoya type of lesions can be found are sickle cell disease, Down syndrome, and neurofibromatosis.

It is found that the vessel constrictions occur as the inner layer of the arteries in cerebrovascular system (arteries that feed the brain structures) begin to "overgrow" inward, slowly narrowing the available area for blood to freely flow. After a while, the vessel becomes so narrow and constricted that it either completely stops the flow of the blood to that area or a clot comes along and forms to finish the job. The result? A stroke...in fact, often there are other strokes that follow the initial insult. Sometimes the progression of the narrowing seems to be unaffected by the use of medical therapy and other methods need to be used.

How is this diagnosed? Usually by some type of imaging method--CT scan, angiogram, or MRI scan. Its name describes what is seen on these imaging studies near the areas where there are vascular constrictions--a "puff of smoke" seems to appear at these areas.

Moyamoya is usually treated by a few methods. Sometimes medications that prevent platelets (the clot forming cells in our blood) from working properly are used. In general, these classes of medications will make the blood harder to clot in hopes of preventing complete blockages at areas that are already constricted. However, the most effective treatments all involve some form or method of vascular surgery near the areas that are found to be blocked. The strategy is to bring a fresh blood supply to the area from outside of the skull from the vascular rich scalp areas to help form new pathways that will eventually bypass around the blood starved areas. In kind of a similar way as how heart vessel bypasses are used to get around clogged areas to help to feed the areas that were not getting much blood any more.

In general, patients who have this disease who are undiagnosed and remain untreated, usually go on to have recurrent strokes and will continue to suffer the consequence of these strokes. It is for this reason that this condition must be diagnosed and treated as soon as possible.

As always, if you have concerns or suspicions that you or someone you know may have these problems, make sure you discuss them with your personal physician.

So until next time....Stay Healthy Hawaii!

Ooops....almost forgot. One Doctor's Opinion will take a short breather for about two weeks...but don't forget to look for it right after that!


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