• Journal of Chest Surgery
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• 제36권10호
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• pp.772-775
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• 2003

모야모야 질환은 원위부 내경 동맥과 근위부 뇌동맥들의 폐쇄성 내피 이형증으로 특징지워는 뇌혈관 질환으로, 아직까지 정확한 병인이 밝혀지지 않은 질환이다. 상기 질환은 양측 내경 동맥의 원위부에 부분적인 협착이나 폐쇄를 야기하게 되고, 이러한 뇌혈관의 해부학적인 변이는 뇌의 기저부에 매우 미세한 혈관들의 문합을 형성하게 하는데, 이러한 신생 혈관들이 뇌혈관 조영검사상 구름처럼 보인다 하여 ‘Moyamoya’라 불리운다. 모야모야 환자군들에게서 관상 동맥 질환 등으로 인한 개심술의 시행은 매우 드물다고 알려져 있으며, 이러한 환자군들에서의 인공심폐기를 이용한 개심술은 수술 중, 그리고 수술 후 허혈성 뇌질환 등의 위험성이 매우 높다고 보고되고 있다. 본 교실은 모야모야 환자에서 심방중격결손 폐쇄와 관상동맥우회술을 인공심폐기를 이용한 개심술을 시행하여 성공적으로 치료하였기에 간략한 문헌 고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.422-427
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• 2015

Moyamoya disease (MMD) is a chronic, progressive, cerebrovascular occlusive disorder that displays various clinical features and results in cerebral infarct or hemorrhagic stroke. Specific genes associated with the disease have not yet been identified, making identification of at-risk patients difficult before clinical manifestation. Familial MMD is not uncommon, with as many as 15% of MMD patients having a family history of the disease, suggesting a genetic etiology. Studies of single nucleotide polymorphisms (SNPs) in MMD have mostly focused on mechanical stress on vessels, endothelium, and the relationship to atherosclerosis. In this review, we discuss SNPs studies targeting the genetic etiology of MMD. Genetic analyses in familial MMD and genome-wide association studies represent promising strategies for elucidating the pathophysiology of this condition. This review also discusses future research directions, not only to offer new insights into the origin of MMD, but also to enhance our understanding of the genetic aspects of MMD. There have been several SNP studies of MMD. Current SNP studies suggest a genetic contribution to MMD, but further reliable and replicable data are needed. A large cohort or family-based design would be important. Modern SNP studies of MMD depend on novel genetic, experimental, and database methods that will hopefully hasten the arrival of a consensus conclusion.

• Clinical and Experimental Pediatrics
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• 제55권11호
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• pp.408-413
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• 2012

Moyamoya disease (MMD) is the most common pediatric cerebrovascular disease in Far Eastern countries. In children, MMD frequently manifests as ischemic symptomatology. Cerebral perfusion gradually decreases as the disease progresses, which often leads to cerebral infarction. The benefits of revascularization surgery, whether direct or indirect, have been well established in MMD patients with ischemic symptoms. In adults, the increase in cerebral blood flow achieved with indirect revascularization is often unsatisfactory, and direct revascularization is usually feasible. In children, however, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. The authors describe surgical procedures and perioperative care in indirect revascularization for MMD. In addition, special considerations with regard to very young patients, patients with recent cerebral infarction, and patients with hyperthyroidism are discussed.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.436-439
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• 2015

The majority of clinical studies on moyamoya disease (MMD) have focused on anterior circulation. The disease involvement of posterior circulation in MMD, mainly in the posterior cerebral artery (PCA), has been mentioned since the early 1980s, and it has been repeatedly emphasized as one of the most important factors related to poor prognosis in MMD. However, its clinical features and outcome have only been elucidated during the last few years. In this review, the angiographic definition of PCA stenosis is summarized. The clinical features are elucidated as being either early-onset or delayed-onset, according to the time of PCA stenosis diagnosis in reference to the anterior circulation revascularization surgeries. The surgical strategy and hypothesis on the mechanism of PCA stenosis is also briefly mentioned. It appears that some MMD patients may show PCA stenosis during the early or late course of the disease and that the presenting symptoms may vary. Because the hemodynamic compromise caused by PCA stenosis may respond well to surgical treatment, clinicians should be aware of the condition, especially during follow-up of MMD patients.

• Journal of Korean Neurosurgical Society
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• 제56권3호
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• pp.188-193
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• 2014

Objective : Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease of unknown etiology. In addition, the neurocognitive impairment of adults with MMD is infrequently reported and, to date, has not been well described. We attempted to determine both the neurocognitive profile of adult moyamoya disease and whether a superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis can improve the neurocognitive impairment in exhibiting hemodynamic disturbance without stroke. Methods : From September 2010 through November 2012, 12 patients with angiographically diagnosed MMD underwent STA-MCA anastomosis for hemodynamic impairment. Patients with hypoperfusion and impaired cerebrovascular reserve (CVR) capacity but without evidence of ischemic stroke underwent a cognitive function test, the Seoul Neuropsychological Screening Battery (SNSB). Five patients agreed to undergo a follow-up SNSB test. Data from preoperative and postoperative neurocognitive function tests were compared and analyzed. Results : Five of 12 patients were enrolled. The median age was 45 years (range, 24-55 years). A comparison of preoperative to postoperative status of SNSB, memory domain, especially delayed recall showed significant improvement. Although most of the domains showed improvement after surgery, the results were not statistically significant. Conclusion : In our preliminary study, large proportions of adult patients with MMD demonstrate disruption of cognitive function. This suggests the possibility of chronic hypoperfusion as a primary cause of the neurocognitive impairment. When preoperative and postoperative status of cognitive function was compared, memory domain showed remarkable improvement. Although further study is needed, neurocognitive impairment may be an indication for earlier intervention with reperfusion procedures that can improve cognitive function.

• 대한장애인치과학회지
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• 제13권1호
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• pp.19-22
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• 2017

비골격성 전방부 개방 교합은 대부분 구강 주위 근육 기능의 부조화로 인한 것이기 때문에 근기능요법을 통한 치료가 추천된다. 그러나 근기능요법의 시행 시에는 환자의 협조도가 필수적이기 때문에, 협조도가 충분하지 못한 경우에는 습관 차단 장치를 적극적으로 활용하는 것이 도움이 될 수 있다. 습관 차단 장치를 단독으로 이용하는 것에 비하여 근기능요법을 병행할 수 있다면 장치 치료의 기간이 단축되고 치료 후의 안정성에도 좋은 결과를 가져올 수 있을 것으로 판단된다.

• Clinical and Experimental Pediatrics
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• 제56권12호
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• pp.545-549
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• 2013

We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left internal carotid artery and right anterior cerebral artery with accompanying collateral circulation (moyamoya vessels). After the patient underwent bypass surgery on the left side, she recovered from the neurological problems and did not experience any additional ischemic attack during the 14-month follow-up period. This case represents an unusual association between moyamoya syndrome and inactive SLE (inactive for a relatively long interval of 2 years) in a young girl.

• Journal of Korean Neurosurgical Society
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• 제62권2호
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• pp.136-143
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• 2019

Moyamoya disease (MMD) is a progressive cerebrovascular disease with unknown etiology, characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki's angiographic staging. Insufficiency of this 'IC-EC conversion system' could result not only in cerebral ischemia, but also in intracranial hemorrhage from inadequate collateral anastomosis, both of which represent the clinical manifestation of MMD. Surgical revascularization prevents cerebral ischemic attack by improving cerebral blood flow, and recent evidence further suggests that extracranial-intracranial bypass could powerfully reduce the risk of re-bleeding in MMD patients with posterior hemorrhage, who were known to have extremely high re-bleeding risk. Although the exact mechanism underlying the hemorrhagic presentation in MMD is undetermined, most recent angiographic analysis revealed the characteristic angio-architecture related to high re-bleeding risk, such as the extension and dilatation of choroidal collaterals and posterior cerebral artery involvement. We sought to update the current management strategy for hemorrhagic MMD, including the outcome of surgical revascularization for hemorrhagic MMD in our institute. Further investigations will clarify the optimal surgical strategy to prevent hemorrhagic manifestation in patients with MMD.

• Journal of Korean Neurosurgical Society
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• 제30권6호
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• pp.800-804
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• 2001

MMD의 외과적 치료에서 소아에서는 직접 및 간접적인 혈관문합술을 병합하거나 아니면 간접적인 혈관문합술들을 병합하여 시행하는 경향이 있고, 성인에서는 혈관의 직경이나 MMD의 진행속도로 보아 STA-MCA 문합술과 같은 직접적인 수술이 많이 활용되고 있으나, 소아나 성인에서나 모두 공통적으로 보다 넓은 뇌표면에 여러 가지 수술방법을 병합하는 것이 좀더 광범위한 범위에 뇌혈류 공급을 증가 시키는데 도움이 된다고 보는 경향이다. 수술의 적응증은 혈관조영상에서의 나타난 질병의 정도보다는 acetazolamide를 이용한 PET 나 SPECT 검사로서 뇌혈류 예비량에 기준을 두고 있는 현상이 혈류 역학적으로도 합리적인 것으로 보인다. 수술후 합병증을 줄이려면 수술중에 정상탄산, 정상체온, 정상혈압을 유지 시킴과 동시에, 수술직후 통증관리도 철저히 하는 것이 매우 중요하다.

• The Journal of Korean Physical Therapy
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• 제25권3호
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• pp.126-131
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• 2013

This case report described a single case of adult stoke patient due to Moyamoya disease through long-term follow-up observation, which included his demographics, brain images, and change of motor function and functional activities. The subject was the 54-year-old male diagnosed with left hemiparesis from a stroke due to multifocal encephalomalacia in both hemispheres. At the time of the stroke attack, he took brain surgery intervention including external ventricular drain. Physical and occupational therapy for stroke rehabilitation were admitted including muscle strengthening exercises, functional activity/ADL training, neurofacilitative techniques with bobath or proprioceptive neuromuscular facilitation concepts, and compensatory strategy. Patient's MRI showed that right frontal lobe, right peri-ventricular area, left parietal, and left occipital lobes were damaged, and MRA showed that abnormal collateral vessel was richly developed in both hemispheres by occlusion of proximal internal carotid arteries in both sides. His motor strength was improved from poor to good grade in all of upper and lower limb motions, that MBC was improved from stage 1 to stage 5. In FAC and barthel index, at the initial evaluation, he could not perform any functional movement, but his FAC and barthel index were on 3 and 14 points at present, respectively. During long-term follow-up for approximately 4 years, the subject's functional motor ability was improved, as similar with recovery progression of usual stroke patient. We believe that this single case report will provide clinical information and concern regarding Moyamoya disease with physical therapist, in terms of such as epidemiology, pathogenesis, diagnostic procedures, clinical features, recovery process, and prognosis.