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A Large Clinical Study on Pediatric Moyamoya Disease (MMD)

Hit : 4,561 Date : 2010-10-12

A Large Clinical Study on Pediatric Moyamoya Disease (MMD)

- The Division of Pediatric Neurosurgery, SNU Children's Hospital, reported a retrospective analysis of the surgical outcome of 410 children with MMDover a period of  20 years.

- Excellent or good outcomes were obtained in 81% of patients after surgical intervention.  

A Koreanmedical team published a result on large-scale clinical research on MMD whichis the most common surgically treated pediatric cerebrovascular disease in East Asia, particularly in Korea and Japan The Division of Pediatric Neurosurgery, SNU Children's Hospital (Professors Kyu-Chang Wang, Seung-Ki Kim, and JiHoon Phi), analyzed clinical characteristics and outcome after surgical treatment of 410 patients with pediatric MMD. According to the study, the symptoms had totally disappeared in 81% of patients going through surgeries.

Patients with cerebral infarction before surgery were three times more likely to suffer from unfavorable clinical outcome. Whereas, patients showing decreased vascular reserve only with normal basal cerebral perfusion  on the SPECT scan were 14 times more likely to have favorable clinical outcome. In addition, the research provides detailed description on digitalized  information of clinical manifestation and  progress after surgery related to MMD.

MMD is a cerebrovascular occlusive disease usually happening in children, in which cerebral blood vessels are gradually occluded, resulting in cerebral infarction or cerebral hemorrhage. Japanese scholars first described the disease and reported it to academic society in 1969. As the shape of small blood vessels growing due to blockage of major cerebral blood vessels looked like rising smoke, the disease was named "Moyamoya" in Japanese. It is particularly common in Korea and Japan. About 90% of its patients around the world are from these two countries but the reason is not known yet.

Research on MMD has been led by Japanese researchers until now. However, MMD treatment was actively carried out and local clinical research on MMD was promoted after the establishment of SNU Children's Hospital in 1985. In 2007 the Research Center for Rare Diseasesin the Ministry for Health, Welfare and Family Affairs was kicked off and basic research on MMD was proactively supported. As a result, many dissertations on clinical and basic research of Korea have been published in international journals.

This research is the largest-scale of all clinical research on pediatric MMD and verifies that the surgical intervention can improve cerebral hemodynamics, reduce further cerebrovascular events and improve long-term outcomes in pediatric MMD. The dissertation is published in the July issue of Annals of Neurology.  

The research team, also, published "Moyamoya Disease Update (Byung-Kyu Cho, Teiji Tominaga eds., Springer, 2010) based on the collection of data on clinical research and basic/ translationalstudies on MMD in cooperation with the research team of Tohoku University, Japan, through Springer, international publisher of scientific books.

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