When we are sick or in pain, it can give us comfort to know why. Many times the answer is clear: if we’re around someone with a cold, we get a cold; if we overdo a certain movement during work or sports, we may develop pain in a limb.
Even with some illnesses that develop due to a combination of factors, such as some types of diabetes, we know that our weight, for example, may play a role.
The roots of some illnesses, however, can be more difficult to explain. For a patient in this situation, finding a cause can bring peace of mind as well as improved treatment.
One example of such an illness is a rare blood vessel disease called Moyamoya. The cause of Moyamoya disease is the subject of a recent study done by neurosurgeons at Columbia University Medical Center/NewYork-Presbyterian Hospital, including Drs. Neal A. Feldstein, Philip M. Meyers and E. Sander Connolly.
Together with Drs. Juan Mejia-Munne (then a medical student) and Jason Ellis (a neurosurgery resident at the time of publication), the Columbia neurosurgeons investigated possible causes of this rare but troubling condition. They published their findings recently in the journal World Neurosurgery.
Moyamoya disease affects the large blood vessels, or arteries, that carry oxygen and nutrients from the heart and lungs to the brain. The arteries become abnormally narrow and may eventually be blocked, cutting off the essential supply of nutrients to the brain.
The deprived brain cells stop working well, which may lead to such symptoms as weakness, paralysis, lack of sensation, speech problems, even seizures. More severe problems, such as bleeding into the brain (hemorrhage) or stroke may result from the blockage.
The body does try to make up for the blockage by growing many small arteries, forming a network of blood vessels that gives the disease its name—blood vessels first described by Japanese researchers as “something hazy, like a puff of cigarette smoke.” The word “Moyamoya” is Japanese for “puff of smoke.”
There’s something else hazy about Moyamoya: The reason the condition occurs has remained unknown, despite the disease’s discovery 50 years ago. That’s where the research of Drs. Feldstein, Meyers and Connolly comes in.
In their journal article, they describe how they gathered data from a large sample of patients admitted to U.S. hospitals over a period of several years. The patients they were most interested in were admitted for stroke or transient ischemic attack (TIA), a more reversible stroke-like illness. Both stroke and TIA are known complications of Moyamoya disease.
Many of the patients had risk factors for atherosclerotic disease. In this condition, a substance called plaque builds up on the walls of arteries, narrowing them. Some general risk factors for atherosclerosis include diabetes, high blood pressure (hypertension) and extra fat in the blood (hyperlipidemia).
Other Moyamoya patients suffered from autoimmune disease. In this family of diseases, the body’s defense system attacks its own tissues. There are many different types of autoimmune disease; some examples are multiple sclerosis, systemic lupus erythematosus (commonly known as “lupus”) and a common type of thyroid disease known as thyroiditis.
How might the presence of atherosclerotic and autoimmune disease be related to Moyamoya disease? First, it’s worth looking at how these two conditions are related to each other. They both involve a process called inflammation.
The word inflammation describes the body’s response to damage. It involves blood cells, chemical substances produced by the body and the blood vessels themselves. For example, when we cut ourselves and see a little redness and swelling around the cut a day or two later, that is an inflammatory response.
Inflammation is useful to protect the body against harmful invaders, such as infections. However, sometimes the body’s inflammatory response goes awry and actually causes disease.
Since atherosclerotic and autoimmune diseases were so frequently found in Moyamoya patients, Drs. Feldstein, Meyers and Connolly concluded that inflammation may play a role in Moyamoya disease as well.
This study takes the striking approach of looking at a large population of patients to try to find clues to what causes their disease. Although it may be one small step in determining the origin of Moyamoya, small steps add up to big discoveries.
As neurosurgeons continue to learn more about why the disease occurs, they will be able to zero in on the most effective treatment. Surely, part of that treatment will be the comfort patients get from knowing more about their illness.
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