Moyamoya disease is a rare cerebrovascular disease that is difficult for even specialist physicians to reach a common conclusion on treatment. However, there are times when surgical treatment promises better results than medication, according to an expert.

"It is a reality that doctors who specialize in treating Moyamoya disease do not have a common conclusion on treatment," said Professor Joo Sung-pil of the Department of Neurosurgery at Chonnam National University Hospital on the YouTube channel with the same name as the hospital. "However, I can say for sure that ischemic Moyamoya disease, which is caused by weakness and lack of blood in the head, can be cured almost 90 percent of the time through surgery."

Moyamoya disease is a rare cerebrovascular disorder for which even experts have difficulty reaching a consensus on treatment, but there are certainly times when surgery promises better outcomes than medication. (Credit: Getty Images)
Moyamoya disease is a rare cerebrovascular disorder for which even experts have difficulty reaching a consensus on treatment, but there are certainly times when surgery promises better outcomes than medication. (Credit: Getty Images)

Moyamoya is a Japanese word that describes the shape of a wisp of smoke. Professor Joo explained that Moyamoya disease is diagnosed when the shape of the brain's blood vessels is inaccurate and blurred through CT, MRI, or angiography. When children suddenly lose strength or young people have brain hemorrhages, one might as well suspect Moyamoya disease, he added.

No specific cause is found in most cases, and about 10 percent of cases have a family history.

"When a young person develops cerebrovascular disease, about 90 percent of the time, there is no specific cause, and the blood vessels in the head are narrowed. When we look at family history, about 10 percent of the time, we find out their parents had Moyamoya disease, or it runs in the family," Professor Joo said.

The most common cases of Moyamoya disease are children around the age of 10 who come to the hospital with severe headaches or weakness when playing school sports or studying, according to Professor Joo. "Symptoms often appear when they are in a situation that causes hyperventilation, gasping for breath. In everyday life, it can be triggered by blowing on hot food,” he said.

In middle-aged and younger women, Moyamoya disease is often caused by a brain hemorrhage.

"Especially in teenagers or young women in their 30s and 40s, the symptoms of Moyamoya disease are severe headaches and sometimes weakness on one side of the body," he said. "After that, it can be diagnosed through tests that can see the blood vessels in the head, such as CT, MRI, and angiography."

"Ischemic” Moyamoya disease, which is a narrowing of the blood vessels that results in poor blood flow, is more likely to be treated with surgery. While surgery is usually more helpful than medication, medication is sometimes recommended.

"If the patient's symptoms are mild or the patient is elderly, surgery may not be helpful, so medication is often used to control the disease, such as nutritional supplements or blood pressure-related conditions," Joo said.

Surgery for Moyamoya disease carries a higher risk of complications than other brain surgeries. "We see complications related to brain surgery in one to three patients out of 100," Professor Joo said. “However, the risk of complications is about five to seven persons out of 100 with Moyamoya disease, twice higher than other brain surgeries."

Joo explained that there is a reason why the risk of complications is higher in Moyamoya disease surgery than in other brain surgeries.

"The reason is that it is a delicate surgery that involves touching the blood vessels of the brain, and it is a disease that is vulnerable to any complications," Professor Joo said. "Even if there is a small complication in the same condition, patients with Moyamoya disease are very sensitive, so more aftereffects are being reported."

 

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