• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.390-395
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• 2015

There have been a few studies reporting the epidemiology of moyamoya disease in Korea. Previous studies revealed relatively high prevalence and incidence of moyamoya disease in Korea and Japan. This study was designed to provide the latest epidemiologic information of moyamoya disease in Korea. We analyzed a database comprising of 50 million people covered in Korea by the National Health Insurance Service to calculate the prevalence. The incidence was estimated by eliminating the duplicated records of previous 3 years. We summarized the prevalence and incidence according to age, sex, and local distribution. In addition, the chronological changes were demonstrated with direct standardization using the 2010 population structure information. The standardized prevalence was 6.5 per 100000 persons in 2005, which was increased to 18.1 in 2013. In the same period, standardized incidence was increased from 2.7 to 4.3 per 100000 persons. The prevalence for men was 4.9 and 8.3 for women in 2005. In 2013, the prevalence had increased for men and women to 13.8 and 25.3, respectively. The incidence for men and women was 2.2 and 3.2, respectively, in 2005. It had increased to 3.5 and 5.7, respectively. The mean age of patients was 33.5 in 2005 and increased to 42.5 in 2013. The peak prevalent age group had shifted slightly to the older age groups, with chronologically consistent female predominance. The prevalence was highest in Jeollabuk province and lowest in Ulsan city.

• Korean Journal of Radiology
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• v.22 no.9
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• pp.1537-1546
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• 2021

Objective: To assess the role of arterial spin-labeling (ASL) perfusion MRI in identifying cerebral perfusion changes after indirect revascularization in children with moyamoya disease. Materials and Methods: We included pre- and postoperative perfusion MRI data of 30 children with moyamoya disease (13 boys and 17 girls; mean age ± standard deviation, 6.3± 3.0 years) who underwent indirect revascularization between June 2016 and August 2017. Relative cerebral blood flow (rCBF) and qualitative perfusion scores for arterial transit time (ATT) effects were evaluated in the middle cerebral artery (MCA) territory on ASL perfusion MRI. The rCBF and relative time-to-peak (rTTP) values were also measured using dynamic susceptibility contrast (DSC) perfusion MRI. Each perfusion change on ASL and DSC perfusion MRI was analyzed using the paired t test. We analyzed the correlation between perfusion changes on ASL and DSC images using Spearman's correlation coefficient. Results: The ASL rCBF values improved at both the ganglionic and supraganglionic levels of the MCA territory after surgery (p = 0.040 and p = 0.003, respectively). The ATT perfusion scores also improved at both levels (p < 0.001 and p < 0.001, respectively). The rCBF and rTTP values on DSC MRI showed significant improvement at both levels of the MCA territory of the operated side (all p < 0.05). There was no significant correlation between the improvements in rCBF values on the two perfusion images (r = 0.195, p = 0.303); however, there was a correlation between the change in perfusion scores on ASL and rTTP on DSC MRI (r = 0.701, p < 0.001). Conclusion: Recognizing the effects of ATT on ASL perfusion MRI may help monitor cerebral perfusion changes and complement quantitative rCBF assessment using ASL perfusion MRI in patients with moyamoya disease after indirect revascularization.

• Journal of Korean Neurosurgical Society
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• v.41 no.5
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• pp.340-342
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• 2007

Three-dimensional computed tomographic angiography [CTA], a representative noninvasive radiologic technique, is being widely used for detecting vascular lesions in specific intracranial bleeding under a certain circumstance [acute nontraumatic subarachnoid hemorrhage]. We encountered a case of extravasation of the contrast medium on CTA images that appeared as ribbon-like high-attenuation lesion from an aneurysm at the distal anterior choroidal artery in a young adult moyamoya patient. As CTA is used more frequently, it is imperative to understand such unusual but, potentially lethal image findings to conduct a prompt intervention.

• Journal of Korean Neurosurgical Society
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• v.54 no.5
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• pp.411-414
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• 2013

The authors present a rare case of adult moyamoya disease in which a patient experienced rapid progression of cerebral infarction after intraventricular hemorrhage (IVH). A healthy 39-year-old woman was admitted to our hospital with sudden headache, a decreased level of consciousness and mild tetraparesis. Initial magnetic resonance imaging revealed small cerebral infarction and IVH. Although the patient underwent conservative therapy including hypervolemia, hemodilution, keeping moderate hypertension and administration of a free radical scavenger, she showed a fulminant clinical course of cerebral infarction. The authors discuss the possible pathophysiology and suggest the treatment for such cases.

• Journal of Chest Surgery
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• v.54 no.2
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• pp.150-153
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• 2021

Moyamoya disease (MMD) is characterized by progressive steno-occlusive lesions of the distal or proximal branch of the internal carotid arteries, and cerebrovascular symptoms are its major complications. Extracranial vascular involvement including the coronary artery has been reported, and some case reports have described variant angina or myocardial infarction. However, no report has yet described a case of myocardial infarction after coronary artery bypass grafting (CABG). Here, we present a patient with MMD who suffered cardiac arrest caused by myocardial infarction due to a coronary spasm after offpump CABG and who was discharged successfully after treatment with a veno-arterial extracorporeal membrane oxygenator and percutaneous coronary intervention.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.1
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• pp.19-22
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• 2017

Moyamoya disease is characterized by progressive bilateral narrowing of the internal carotid arteries(ICA) and their main branches, and formation of a fine vascular network (moyamoya vessels) at the base of brain. Mild intellectual or motor impairment, or both can be seen in moyamoya disease patient. Patients having intellectual disability usually have malocclusions associated with oral habits such as tongue thrusting, bruxism, or clenching. A patient with moyamoya disease and cerebral palsy visited Chonbuk National University hospital for dental examination. She showed mild openbite on anterior teeth, and had oral habits such as mouth breathing, tongue thrusting, and drooling. She was treated with myofunctional therapy(MFT) whose purpose was to strengthen orofacial muscles. Lip closing force and bite force were recorded to evaluate the muscle tone. Tongue crib was delivered to restrict tongue thrusting habit, since her compliance to the MFT was decreased after 6 months of treatment. With the MFT and tongue crib, the openbite was fairly improved. When myofunctional therapy has difficulty in obtaining good compliances, the treatment should not be effective enough. Although intellectually disabled patients are not compliant enough, thus a habit breaking device should be needed to treat the openbite caused by oral habits, dentist should make an effort to do MFT, since it could reduce a treatment period, and enhance the stability.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.294-298
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• 2007

Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hyper tension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, bat-loon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.

• The Korean Journal of Nuclear Medicine
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• v.28 no.1
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• pp.22-30
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• 1994

Encephalo-duro-arterio-synangiosis (EDAS) is a relatively new surgical procedure for treatment of childhood moyamoya disease. We assessed regional cerebral perfusion in moyamoya patients before (1.3 mo) and after (6.8 mo) EDAS with $^{99m}Tc$-HMPAO brain SPECT. A total of 21 EDAS operations in 17 moyamoya patients was included. Preoperative CT or MRI showed cerebral infarction in 14 patients and carotid angiography showed Suzuki grade I to V stenosis in 6%, 9%, 62%, 12% and 12% of the hemispheres respectively. Preoperative SPECT showed regional hypoperfusion in all patients, bilateral frontal and temporal lobes being the most frequently involved site. $4{\times}4$ pixel sized ROIs were applied on the frontotemporal cortex in 3 slice averaged transverse tomographic images. An index of regional perfusion was measured as: PI (%)=average F-T activity/average cerebellar activity${\times}100$ Pre-EDAS ipsilateral PI ranged from 23.7 to 98.4% (mean: $74.3{\pm}17%$) and increased significantly after operation ($81.4{\pm}17%$, p<0.001). Individual post-EDAS PI improved in 15/21 cases, showed no significant change in 5 and was slightly aggravated in 1. The amount of clinical improvement (${\Delta}CI$) was graded with a scale of 0 to 4 based on frequency and severity of TIA attacks. When patients were grouped according to pre-EDAS PI, group II (PI 70-89) showed a significantly higher ${\Delta}CI$ (3.3) compared to group I (PI< 70, 1.57) or group III (PI >90, 0.5) (P< 0.001). The amount of perfusion improvement (${\Delta}PI$) showed significant correlation with ${\Delta}CI$ (r=0.42, p=0.04). ${\Delta}PI$ did not, however, correlate with the amount of neovascularization assessed angiographically in 8 patients. Serial HMPAO SPECT is an useful noninvasive study for assessing perfusion improvement after EDAS in childhood moyamoya patients.

• Journal of Korean Neurosurgical Society
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• v.30 no.6
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• pp.800-804
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• 2001

The authors reviewed the recent trend of surgical treatment for pediatric as well as adult onset moyamoya disease(MMD). Combined direct and indirect arterial anastomosis or multiple indirect arterial bypasss has been increasing for pediatric MMD and direct arterial bypass is recommended for adult MMD, especially in patients with hemorrhagic MMD. Besides perioperative complications related to the management of MMD and 1999 annual report by the Research Committee on Spontaneous Occlusion of the Circle of Willis(Moyamoya Disease) of the Ministry of Health and Welfare, Japan will be summarizd.

• The Journal of Korean Physical Therapy
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• v.25 no.3
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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.