• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.408-414
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• 2015

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

• Journal of Korean Neurosurgical Society
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• v.48 no.1
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• pp.8-13
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• 2010

Objective : The etiology and pathogenesis of moyamoya disease remain unclear. Furthermore, the definitive diagnostic protein-biomarkers for moyamoya disease are still unknown. The present study analyzed serum proteomes from normal controls and moyamoya patients to identify novel serological biomarkers for diagnosing moyamoya disease. Methods : We compared the two-dimensional electrophoresis patterns of sera from moyamoya disease patients and normal controls and identified the differentially-expressed spots by matrix-assisted laser desorption/ionization-time-of flight mass spectrometry and electrospray ionization quadruple time-of-flight mass spectrometry. Results : We found and analyzed 22 differently-expressed proteomes. Two proteins were up-regulated. Twenty proteins were down-regulated. Complement C1 inhibitor protein and apolipoprotein C-III showed predominantly changed expressions (complement C1 inhibitor protein averaged a 7.23-fold expression in moyamoya patients as compared to controls, while apolipoprotein C-III averaged a 0.066-fold expression). Conclusion : Although our study had a small sample size, our proteomic data provide serologic clue proteins for understanding moyamoya disease.

• Clinical and Experimental Pediatrics
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• v.57 no.6
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• pp.287-291
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• 2014

Moyamoya disease is a cerebrovascular condition that results in the narrowing of the vessels of the circle of Willis and collateral vessel formation at the base of the brain. Although relationships between Graves' disease and cerebrovascular accidents in Moyamoya disease are obscure, the coexistence of the two diseases is noteworthy. Moyamoya disease has been rarely reported in adolescent patients with thyrotoxicosis. Recently, we encountered two adolescent Korean patients with Moyamoya disease associated with Graves' disease who presented with episodic right-sided hemiparesis and syncope. These two girls who had Graves' disease had no history of other diseases or head trauma. A thyroid function test revealed a euthyroid state and a high thyroid-stimulating hormone (TSH) receptor antibody titer at that time. The patients were diagnosed with Moyamoya disease based on brain magnetic resonance angiography and cerebral four-vessel angiography. The patients underwent cranial revascularization by encephalo-duroarterio-synangiosis as soon as a diagnosis was made, which resulted in successful symptom resolution. They fared well and had no additional neurological symptoms as of their last follow-up visits. Here, we report these two cases of confirmed Moyamoya disease complicated by Graves' disease with a review of the literature, and discuss the possible association between the two diseases. To our knowledge, this is the first report in South Korea on Moyamoya disease associated with Graves' disease in adolescents with a euthyroid.

• Journal of Korean Neurosurgical Society
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• v.50 no.3
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• pp.256-259
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• 2011

We report a rare case of Williams syndrome accompanying moyamoya disease in whom postoperative global cerebral infarction occurred unpredictably. Williams syndrome is an uncommon hereditary disorder associated with the connective tissue abnormalities and cardiovascular disease. To our knowledge, our case report is the second case of Williams syndrome accompanying moyamoya disease. A 9-year-old boy was presented with right hemiparesis after second operation for coarctation of aorta. He was diagnosed as having Williams syndrome at the age of 1 year. Brain MRI showed left cerebral cortical infarction, and angiography showed severe stenosis of bilateral internal carotid arteries and moyamoya vessels. To reduce the risk of furthermore cerebral infarction, we performed indirect anastomosis successfully. Postoperatively, the patient recovered well, but at postoperative third day, without any unusual predictive abnormal findings the patient's pupils were suddenly dilated. Brain CT showed the global cerebral infarction. Despite of vigorous treatment, the patient was not recovered and fell in brain death one week later. We suggest that in this kind of labile patient with Williams syndrome accompanying moyamoya disease, postoperative sedation should be done with more thorough strict patient monitoring than usual moyamoya patients. Also, we should decide the revascularization surgery more cautiously than usual moyamoya disease. The possibility of unpredictable postoperative ischemic complication should be kept in mind.

• Journal of Korean Neurosurgical Society
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• v.42 no.1
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• pp.49-52
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• 2007

The authors report a unique case of unilateral Moyamoya disease with a rare combination of arteriovenous malformation (AVM) who presented with intracerebral hemorrhage (ICH). A 50-year-old man suffered from sudden onset of mental deterioration and right hemiparesis. Brain computed tomography (CT) showed intracerebral hemorrhage on left thalamus. Brain CT angiography and cerebral digital subtraction angiography (DSA) revealed AVM combined with unilateral moyamoya disease involving left middle cerebral artery (MCA) and choroid plexus in left lateral ventricle. Intraventricular hemorrhage and hydrocephalus were managed conservatively. A rare case of unilateral Moyamoya disease accompanied by a cerebral arteriovenous malformation is described and discussed with review of pertinent literature.

• Journal of Neurocritical Care
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• v.11 no.2
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• pp.129-133
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• 2018

Background: A few cases of moyamoya syndrome associated with thyrotoxicosis have been reported. However, studies on the association of hyperthyroidism with moyamoya syndrome are insufficient. Case Report: Here we report a case of hyperthyroidism associated with moyamoya syndrome in a 41-year-old woman with aphasia and right side weakness. Brain imaging revealed acute cerebral infarction of left middle cerebral artery territory and occlusion of bilateral distal internal carotid arteries. Conclusion: Antithyroid medication stabilized the patient's neurologic deterioration, suggesting that thyrotoxicosis could aggravate acute cerebral infarction caused by moyamoya syndrome.

• Journal of Korean Neurosurgical Society
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• v.37 no.3
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• pp.207-212
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• 2005

Objective: Classically, single photon emission tomography is known to be the reference standard for evaluating the hemodynamic status of patients with moyamoya disease. Recently, T2-weighted perfusion magnetic resonance(MR) imaging has been found to be effective in estimating cerebral hemodynamics in moyamoya disease. We aim to assess the utility of perfusion-weighted MR imaging for evaluating hemodynamic status of moyamoya disease. Methods: The subjects were fourteen moyamoya patients(mean age: 7.21 yrs) who were admitted at our hospital between Sep. 2001 to Sep 2003. Four normal children were used for control group. Perfusion MR imaging was performed before any treatment by using a T2-weighted contrast material-enhanced technique. Relative cerebral blood volume(rCBV) and time to peak enhancement(TTP) maps were calculated. Relative ratios of rCBV and TTP in the anterior cerebral artery(ACA), middle cerebral artery(MCA) and basal ganglia were measured and compared with those of the posterior cerebral artery(PCA) in each cerebral hemispheres. Using this data, we analysed the hemodynamic aspect of pediatric moyamoya disease patients in regarding to the age, Suzuki stage, signal change in FLAIR MR imaging, and hemispheres inducing symptoms. Results: The mean rCBV ratio of ACA, MCA did not differ between normal children and moyamoya patients. However the significant TTP delay was observed at ACA, MCA territories (mean = 2.3071 sec, 1.2089 see, respectively, p < 0.0001). As the Suzuki stage of patients is advanced, rCBV ratio is decreased and TTP differences increased. Conclusion: Perfusion MR can be applied for evaluating preoperative cerebral hemodynamic status of moyamoya patients. Furthermore, perfusion MR imaging can be used for determine which hemisphere should be treated, first.

• Journal of Korean Neurosurgical Society
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• v.50 no.6
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• pp.535-537
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• 2011

Recently, combination of surgery and radiation therapy (RT) has been recommended in the treatment of craniopharyngioma. RT could be associated with late complications, including vasculopathy. We report two cases of the moyamoya syndrome seen in children with craniopharyngioma who received RT after surgical resection. Thirty-five patients in pediatric age with craniopharyngioma were surgically treated. Fifteen out of 35 patients underwent surgical resection followed by RT or gamma knife surgery. Two of the 15 were found to have symptoms of transient ischemic attack and were diagnosed as moyamoya syndrome through the cerebral angiography. Age at RT was 4 and 13 years, respectively. The latent period for development of the moyamoya syndrome was 27 months and 3 years, respectively, after RT. The RT dose of both patients was 54 Gy. These two patients received bilateral encephaloduroarteriosynangiosis procedures. We report here these two cases of radiation-induced moyamoya syndrome in pediatric craniopharyngioma. Pediatric patients with craniopharyngioma who received RT should be reminded, during follow-up, about the risk of development of the moyamoya syndrome.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.440-444
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• 2015

Quality of life is the current trend and issue for the most of human diseases. In moyamoya disease (MMD), surgical revascularization has been recognized as the possible assistance to reduce the neurological insult. However, the progressive nature of the disease has been invincible so far. To improve the quality of life of MMD patients not only the protection from the neurological insult but also the maintenance or improvement of cognitive function is inevitable. For pediatric MMD patients, younger age or longer duration of disease is the key factor among the prognostic factors for bad neurological outcomes. Hence, 'the earlier, the better' is the most precious rule for treatment. Protection from neurological insult is very critical and foremost important to improve cognitive outcome. Clinicians need to know the neuropsychological profile of MMD patients for the care of whole person and make an effort to protect the patients from neurological insults to maintain or improve it.

• 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.