• 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.

• Journal of Korean Neurosurgical Society
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• 제46권6호
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• pp.558-563
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• 2009

Objective : Moyamoya disease (MMD) is an uncommon cerebrovascular disorder, characterized by progressive occlusion at the terminal portion of the internal carotid artery. Incidence of the disease is high in East Asia and familial MMD accounts for about 15% of the disease. Although the pathogenesis is unknown, association of HLA class I or II alleles with MMD has been reported with conflicting results. We investigated whether there is a difference in HLA class II association between familial and non-familial forms of the disease. Methods : A total of 70 Korean children with MMD, including 16 familial cases (10 probands), and 207 healthy controls were studied. Among familial cases, only 10 probands were used for the HLA frequency analysis. High resolution HLA-DRB1 and DQB1 genotyping was performed using polymerase chain reaction (PCR)-sequence specific oligonucleotide hybridization and PCR-single strand conformation polymorphism methods. Results : The phenotype frequencies of HLA-DRB1*1302 (70.0%) and DQB1*0609 (40.0%) were significantly increased in familial MMD compared to both controls [vs. 15.5%, corrected p ($p_c$) = 0.008, odds ratio (OR) = 12.76; vs. 4.3%, $p_c\;=\;0.02$, OR = 14.67] and non-familial MMD patients (vs. 14.8%, $p_c\;=\;0.02$, OR = 13.42; vs. 1.9%, $p_c\;=\;0.02$, OR = 35.33). The frequencies of DRB1 and DQB1 alleles in non-familial MMD patients were not significantly different from those in controls. Conclusion : Our findings suggest that the genetic polymorphism of HLA class II genes or other closely linked disease relevant gene(s) could be a genetic predisposing factor for familial MMD.

• Journal of Korean Neurosurgical Society
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• 제41권5호
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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.

• Clinical and Experimental Pediatrics
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• 제56권12호
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• pp.540-544
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• 2013

Down syndrome, the most common chromosomal abnormality, may be associated with various neurologic complications such as moyamoya syndrome, cervical spinal cord compression due to atlantoaxial subluxation, and basal ganglia damage, as well as epileptic seizures and stroke. Many cases of Down syndrome accompanied by isolated neurologic manifestations have been reported in children; however, Down syndrome with multiple neurologic conditions is rare. Here, we have reported a case of Down syndrome in a 10-year-old girl who presented with asymptomatic moyamoya syndrome, atlantoaxial subluxation with spinal cord compression, and basal ganglia calcification. To the best of our knowledge, this is the first report of Down syndrome, in a child, which was accompanied by these 3 neurologic complications simultaneously. As seen in this case, patients with Down syndrome may have neurologic conditions without any obvious neurologic symptoms; hence, patients with Down syndrome should be carefully examined for the presence of neurologic conditions.

• 대한장애인치과학회지
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• 제15권1호
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• pp.40-44
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• 2019

본 증례는 다수의 치아우식증을 주소로 내원한 모야모야병 환자의 전신마취 하 치과치료에 대한 보고이다. 모야모야병은 치과치료 동안 상당히 주의를 필요로 하는 다양한 전신질환과 관련이 있다. 여러 과의 의사들과 협진이 필요하고, 치과 예방치료에 초점을 맞추면서 적절한 시기에 치료하는 것이 중요하다. 모야모야 환자에서 울음과 과호흡은 저칼륨혈증을 일으킬 수 있고, 대뇌 혈관 수축 효과를 일으킬 수 있다. 치과치료 시 뇌졸중 발생을 예방하기 위해서 통증과 불안을 조절하는 것이 매우 중요하다. 비협조적이거나 매우 어린 모야모야병 환자에게 치과치료를 하기 위해서는 전신마취가 필요할 수 있다.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.143-148
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• 2013

신경섬유종은 드문 전신 질환으로 여러 장기를 침범하며 특히 다양한 크기의 혈관을 침범하여 혈관병을 발생시켜 대동맥 협착, 모야모야병, 신동맥 협착 등을 일으킨다. 이로 인하여 약 0.4-6.4%의 환자에서 고혈압 증상이 있으며 이의 원인으로 신혈관 협착이 가장 흔하다. 특히 약물에도 조절되지 않는 고혈압은 혈관 협착 등이 원인이 될 수 있기 때문에 도플러 초음파 검사나 전산화 혈관 조영술 등의 정밀 검사가 필요하다. 이에 대한 치료는 약물 치료, 피부경유 혈관경유혈관성형술, 수술적 치료 등의 병합 요법으로 이루어지며 재협착 발생률이 높기 때문에 주의 깊은 추적 관찰이 필요하다. 본 저자들은 제 1형 신경섬유종증에 합병된 모야모야병에서 신장동맥 협착을 동반한 고혈압을 진단받고 치료한 1례를 경험하였기에 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.401-407
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• 2015

The nonprogressive unilateral intracranial arteriopathy known as transient (focal) cerebral arteriopathy is not a well-recognized arteriopathy among practitioners of Korea and Japan, although it cannot be easily differentiated from early moyamoya disease. This review summarizes the nomenclature, pathophysiology, diagnostic evaluation, clinico-radiological features, and management of nonprogressive (reversible or stable) unilateral arteriopathy based on the relevant literature and our own experiences. Nonprogressive unilateral arteriopathy should be strongly suspected in children presenting with basal ganglia infarction and arterial beading. The early identification of patients likely to have nonprogressive or progressive arteriopathy would ensure proper management and guide further research for secondary stroke prevention.

• 대한자기공명의과학회:학술대회논문집
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• 대한자기공명의과학회 2000년도 제5차 추계학술대회
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• pp.28-28
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• 2000

• 대한방사선기술학회지:방사선기술과학
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• 제30권4호
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• pp.427-433
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• 2007

모야모야 질병에 있어서 펄스 동맥 스핀표지 영상기법을 이용한 뇌혈류량 영상과 고식적 관류 자기공명 영상을 전대뇌동맥, 중대뇌동맥, 후대뇌동맥을 좌우 6개 영역으로 나누어 시각적 평가와 정량적 평가를 하여 펄스 동맥 스핀표지 영상기법의 유용성을 알아보고자 하였다. 시각적인 평가에서는 펄스 동맥 스핀표지 혈류량 영상과 고식적인 관류 자기공명 뇌혈류량 영상에서 관류가 감소된 범위가 자기공명 혈관조영술에서 결손된 위치와 일치하였다. 정량적인 영상 평가는 펄스 동맥 스핀표지 뇌혈류량 영상과 고식적인 관류 자기공명 뇌혈류량 영상에서 관류가 감소된 범위와 위치는 일치하였지만 혈류의 양을 추정할 수 있는 평가에서는 다소간의 차이를 보였다.