• Journal of Korean Neurosurgical Society
• /
• 제57권6호
• /
• pp.390-395
• /
• 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
• /
• 제22권9호
• /
• pp.1537-1546
• /
• 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
• /
• 제41권5호
• /
• pp.340-342
• /
• 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
• /
• 제54권5호
• /
• pp.411-414
• /
• 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
• /
• 제54권2호
• /
• pp.150-153
• /
• 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.

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

• Childhood Kidney Diseases
• /
• 제11권2호
• /
• pp.294-298
• /
• 2007

모야모야병은 주로 뇌혈관 협착을 유발하여 측부순환을 생성하는 질환으로 최근 뇌혈관 이외의 혈관에도 침범할 수 있음이 보고되고 있다. 지금까지 모야모야병에서 폐동맥, 관상동맥, 말초동맥 및 신동맥의 협착을 동반한 증례들이 발표되고 있다. 이 중 신동맥 협착을 동반한 경우 이로 인한 신성고혈압을 보이는 증례들이 보고되고 있으며 이는 모야모야병에서 지속적인 고혈압을 보여 2차적인 뇌경색이나 뇌출혈 등의 합병증을 유발할 수 있으므로 모야모야 환자의 치료에 있어서 항상 면밀하게 평가되어야 할 부분이다. 저자들은 모야모야병으로 양측 뇌경질막동맥간접문합술을 시행 받고 경과관찰 하던 환아에서 지속되는 고혈압으로 인해 발견된 신동맥 협착 및 이로 인한 신성 고혈압을 경험하였기에 이를 보고하는 바이다.

• 대한핵의학회지
• /
• 제28권1호
• /
• pp.22-30
• /
• 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
• /
• 제30권6호
• /
• pp.800-804
• /
• 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
• /
• 제25권3호
• /
• pp.126-131
• /
• 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.