• Title/Summary/Keyword: 모야모야병

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Perfusion RRI of the Brain Using Oxygen Inhalation (산소 호흡을 이용한 뇌의 관류 자기공명영상)

  • 최순섭
    • Investigative Magnetic Resonance Imaging
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    • v.4 no.2
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    • pp.113-119
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    • 2000
  • Purpose : To know the possibility of clinical application of MRI using oxygen inhalation as a perfusion MRI Materials and methods : Two healthy volunteers and three patients of one moyamoya disease, one acute infarction and one meningioma were studied using a 1.5 Tesla MRI unit. Oxygen (15 liters/min) mixed with room air was given using face mask from 8 second to 35 second during the study. Images were acquired 25 times (scan time per study were 1.6 seconds) using susceptibility contrast EPI (echo planar image) sequence. Difference maps were acquired by early (study 12-18), and late (study 19-25) O2 inhalation image groups minus pre-O2 inhalation image group (study 3-9) with a Z-score of 0.7-1.0 using VB31C program of Magneton Vision. The resulting perfusion images were created by superimposition of difference maps on corresponding T1 weighted anatomic images. On moyamoya patient, similar perfusion images were acquired after Gd-DTPA injection, and compared with O2 inhalation perfusion images. Results ; The author can get the perfusion images of the brain by oxygen inhalation with susceptibility contrast EPI sequence at the volunteers, and the patient of moyomoya disease, acute infarction and meningioma. On moyamoya patient, perfusion images with O2 inhalation are similar with perfusion images by Gd-DTPA injection. Conclusion 1 This study has demonstrated that the susceptibility contrast EPI by oxygen inhalation can be used as the clinically useful perfusion MRI technique

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Factors Influencing Health Behavior Compliance in Adult Moyamoya Patients (성인 모야모야병 환자의 건강행위 이행에 영향을 미치는 요인)

  • Kim, Bo Eun;Song, Ju-Eun
    • Journal of Korean Academy of Nursing
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    • v.51 no.1
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    • pp.80-91
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    • 2021
  • Purpose: The purpose of this study was to identify factors influencing health behavior compliance in adult patients with moyamoya. Methods: A descriptive correlation study was conducted to investigate the factors influencing health behavior compliance. Participants were 142 adult patients diagnosed with moyamoya disease who were hospitalized or visited an outpatient clinic in the Gyeonggi province. Data were collected from December 16, 2019 to April 14, 2020 using self-report questionnaires and analyzed using the IBM SPSS 26.0 Win software. Results: The hierarchical multiple regression analysis demonstrated that self-efficacy (β = .60, p < .001), social support (β = .13, p = .032), and age (β = .21, p = .005) affected the health behavior of adults with moyamoya disease. These 3 variables explained 62.0% of the variance of health behavior compliance, and the most influential factor was self-efficacy. Conclusion: Based on the results of this study, it concludes that nursing interventions should be focused on self-efficacy and social support to improve health behavior compliance with adult patients diagnosed with moyamoya disease. For that, various strategies to enhance self-efficacy and social support should be developed and actively applied in the clinical setting for adult moyamoya patients.

DENTAL MANAGEMENT OF A PATIENT WITH MOYAMOYA DISEASE UNDER GENERAL ANESTHESIA: CASE REPORT (모야모야병(moyamoya disease) 환자의 전신마취 하 치과치료: 증례보고)

  • Chae, Jong Kyun;Song, Ji-Soo;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Kim, Young-Jae
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.15 no.1
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    • pp.40-44
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    • 2019
  • Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease of unknown etiology characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. The clinical presentations of MMD include transient ischemic attacks (TIA), ischemic stroke, hemorrhagic stroke, seizures, headache, and cognitive impairment. MMD is the most important cause of stroke or TIA in children in East Asian countries. A 5-year-3-month old boy with MMD experienced cerebral infarctions five times. Cerebrovascular anastomosis surgery was performed on him four years ago. He had dysphagia, developmental delay, hemiplegia, and strabismus. Besides, a number of dental caries in primary dentition were identified during clinical oral examination. Dental treatment under general anesthesia using sevoflurane was performed due to his lack of cooperation and underlying systemic disease. MMD is associated with various medical diseases requiring thoughtful consideration during dental treatment. Crying and hyperventilation in MMD patients may cause hypocapnia and have a cerebral vasoconstrictive effect. If dental treatment is required, control of pain and anxiety is very important. General anesthesia may be considered for dental treatment in uncooperative or very young patients with MMD.

Analysis of hemodynamics in cerebral artery related to moyamoya disease (모야모야병과 연관된 뇌동맥에서의 혈류역학 분석)

  • Lee, Seung-Cheol;Lim, Ki-Moo;Shim, Eun-Bo
    • Proceedings of the KSME Conference
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    • 2008.11a
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    • pp.1647-1650
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    • 2008
  • The moyamoya disease is a type of cerebrovascular disease which produces thin abnormal blood vessels like haze in the brain base because the end of internal carotid artery which supplies about 80% of blood is blocked. Regarding this moyamoya disease, the shearing stress and thrombus generation are mentioned as its main causes. This study three-dimensionally implemented the ICA, ACA, and MCA parts of the cerebrovascular configuration related to the moyamoya disease, and analyzed the hydrodynamic phenomenon with the commercial program ADINA. In particular, the correlations between shearing stress and speed distribution according to the branch angle of ACA and MCA. A numerical analysis found that the greater the branch angle of ACA and MCA, the lower the shearing stress and the greater the stationary area of the flow.. Put Abstract text here.

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Evaluation of Surgical Outcome with Pre-and Post-operative Rest/Acetazolamide Tc-99m HMPAO SPECT in Children with Moyamoya Disease (어린이 모야모야병에서 휴식/아세타졸아미드 Tc-99m-HMPAO SPECT를 이용한 수술결과 평가)

  • Lee, D.S.;Hyun, I.Y.;Wang, K.C.;Cho, B.K.;Chung, J.K.;Lee, M.C.
    • The Korean Journal of Nuclear Medicine
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    • v.32 no.4
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    • pp.314-324
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    • 1998
  • Purpose: This study was performed to evaluate the outcome of encephalo-duro-arterio-synangiosis (EDAS) surgery with rest/acetazolamide Tc-99m HMPAO SPECT in moyamoya disease. Materials and Methods: Rost/acetazolamide subtraction SPECT with consecutive acquisition were done before and 2 months after 21 EDAS surgeries in 18 patients. Perfusion decrease was graded visually for 14 areas of each hemisphere as 0 (normal) to 3 (defect) using 4 point scoring system. Postoperative rest perfusion or perfusion reserve was compared with preoperative ones. Results: Among 294 areas of 21 hemispheres, rest perfusion abnormality was found in 91 areas of 15 hemispheres. Decrease of perfusion reserve was found in 146 areas of 18 hemispheres. Six hemispheres having normal rest perfusion and 12 of 15 hemispheres having rest perfusion abnormality showed reserve decrease. Three having rest perfusion defect did not change after acetazolamide in preoperative SPECT. After operation, 16 patients (89%) demonstrated clinical improvement. Fifteen among 18 hemispheres (83%) with decreased reserve improved. Rest perfusion abnormality improved in 6 among the 15 hemispheres (40%). The areas having rest perfusion and/or reserve decrease improved in 87 among 146 areas (60%). Decrease of reserve, improved in 85% (68/80). However, areas without reserve decrease also improved in 29% (19/66). The better was preoperative rest perfusion in involved areas or the more decreased vascular reserve, the more improved perfusion and reserve after operation. Conclusion: We conclude that assessment of perfusion and Perfusion reserve using rest/acetazolamide brain perfusion SPECT predict the surgical outcome in patients with moyamoya disease.

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A Case of Moyamoya Disease (모야모야병 환아(患兒) 1예(例)에 대한 증례(症例))

  • Yu Sun-Yae;Lee Seung-Yeon
    • The Journal of Pediatrics of Korean Medicine
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    • v.14 no.2
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    • pp.141-147
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    • 2000
  • Moyamoya is a chronic cerebrovascular disease chracterized by progressive stenosis or occlusion of the terminal parts of both internal carotid arteries with telangiectatic vascular network of collateral circuration at the base of the brain and leptomeningeal arteries. The etiology and pathophysiology of this disease are still unknown. The clinical course in those whose first symptoms occur in childhood is different from those in whom symptoms develop in adult life. The term moyamoya disease should be resserved for those cases in which the chracteristic angiographic pattern is idiopathic; moayamoya syndrome is used when the underlying condition is known. we have experienced a case of moyamoya syndrome in a 5-year-2-month-old boy who presented right-sided hemiparesis. A cerebral angiogram revealed occlusion of abnomal collateral network. Moyamoya disease is applicable to stroke of an infant from oriental medicine point of view, and The symptoms is similar to adult stroke, we have treated adult stroke patint with herb medicine and acupuncutre and physical treatment. The acute stage of stroke is applied to the external treatment(標治), and The recovery stage is applied to the basic treament(本治).

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A Case of Moyamoya Disease with Neurofibromatosis Type I (제 1형 신경섬유종증에 합병된 모야모야병 1례)

  • Lee, Mi A;Eom, Joo Pil;Lee, Hae Young;Cha, Byung Ho
    • Clinical and Experimental Pediatrics
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    • v.48 no.1
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    • pp.93-96
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    • 2005
  • Neurofibromatosis type I is an autosomal dominant disorder with varied manifestations in bone, soft tissue, the nervous system and skin. This is characterized by cafe-au-lait spots, neurofibromas, Lisch nodules, optic glioma, bony displasia, and intertriginous freckling. One of the more serious aspect of the disease relates to the arterial involvement. Vascular changes in neurofibromatosis may occur in any arterial tree from the proximal aorta to the small arteries but these changes are most common in the renal arteries, aorta, celiac arteries and mesenteric arteries. Of the many complications observed in neurofibromatosis type I, cerebrovascular lesions may be the least appreciated. About 40 cases of neurofibromatosis type I associated with occlusive cerebrovascular disorders have been reported in the literature, but MRI and angiographic findings typical of moyamoya disease are rarely described. We experienced a case of moyamoya disease associated with neurofibromatosis type I in a 3-year-old girl who of complained gait disturbance and paraparesis and showed findings typical of moyamoya disease on MRI and carotid angiogram.

A Case of Moyamoya Disease Associated with Neurofibromatosis Type 1 in Patients with Renal Artery Stenosis and Hypertension (제 1형 신경섬유종증에 합병된 모야모야병에서 신장동맥 협착을 동반한 고혈압)

  • Seo, Young Ho;Yim, Hyung Eun;Yoo, Kee Hwan
    • Childhood Kidney Diseases
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    • v.17 no.2
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    • pp.143-148
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    • 2013
  • Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.

Assessment of Cerebral Hemodynamic Changes in Pediatric Patients with Moyamoya Disease Using Probabilistic Maps on Analysis of Basal/Acetazolamide Stress Brain Perfusion SPECT (소아 모야모야병에서 뇌확률지도를 이용한 수술전후 혈역학적 변화 분석)

  • Lee, Ho-Young;Lee, Jae-Sung;Kim, Seung-Ki;Wang, Kyu-Chang;Cho, Byung-Kyu;Chung, June-Key;Lee, Myung-Chul;Lee, Dong-Soo
    • Nuclear Medicine and Molecular Imaging
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    • v.42 no.3
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    • pp.192-200
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    • 2008
  • To evaluate the hemodynamic changes and the predictive factors of the clinical outcome in pediatric patients with moyamoya disease, we analyzed pre/post basal/acetazolamide stress brain perfusion SPECT with automated volume of interest (VOIs) method. Methods: Total fifty six (M:F = 33:24, age $6.7{\pm}3.2$ years) pediatric patients with moyamoya disease, who underwent basal/acetazolamide stress brain perfusion SPECT within 6 before and after revascularization surgery (encephalo-duro-arterio-synangiosis (EDAS) with frontal encephalo-galeo-synangiosis (EGS) and EDAS only followed on contralateral hemisphere), and followed-up more than 6 months after post-operative SPECT, were included. A mean follow-up period after post-operative SPECT was $33{\pm}21$ months. Each patient's SPECT image was spatially normalized to Korean template with the SPM2. For the regional count normalization, the count of pons was used as a reference region. The basal/acetazolamide-stressed cerebral blood flow (CBF), the cerebral vascular reserve index (CVRI), and the extent of area with significantly decreased basal/acetazolamide- stressed rCBF than age-matched normal control were evaluated on both medial frontal, frontal, parietal, occipital lobes, and whole brain in each patient's images. The post-operative clinical outcome was assigned as good, poor according to the presence of transient ischemic attacks and/or fixed neurological deficits by pediatric neurosurgeon. Results: In a paired t-test, basal/acetazolamide-stressed rCBF and the CVRI were significantly improved after revascularization (p<0.05). The significant difference in the pre-operative basal/acetazolamide-stressed rCBF and the CVRI between the hemispheres where EDAS with frontal EGS was performed and their contralateral counterparts where EDAS only was done disappeared after operation (p<0.05). In an independent student t-test, the pre-operative basal rCBF in the medial frontal gyrus, the post-operative CVRI in the frontal lobe and the parietal lobe of the hemispheres with EDAS and frontal EGS, the post-operative CVRI, and ${\Delta}CVRI$ showed a significant difference between patients with a good and poor clinical outcome (p<0.05). In a multivariate logistic regression analysis, the ${\Delta}CVRI$ and the post-operative CVRI of medial frontal gyrus on the hemispheres where EDAS with frontal EGS was performed were the significant predictive factors for the clinical outcome (p =0.002, p =0.015), Conclusion: With probabilistic map, we could objectively evaluate pre/post-operative hemodynamic changes of pediatric patients with moyamoya disease. Specifically the post-operative CVRI and the post-operative CVRI of medial frontal gyrus where EDAS with frontal EGS was done were the significant predictive factors for further clinical outcomes.