• 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.

• 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 Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.108-113
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• 2017

Moyamoya disease is a disorder in which certain arteries in the brain are constricted. Blood flow can be blocked by the constriction and blood clots. The patients frequently experience transient ischemic attacks (TIA), cerebral hemorrhage, or may not experience any symptoms at all. It is reported that they have a higher risk of recurrent stroke and a distinct underlying pathophysiology. A 3-year-8-month old boy with moyamoya disease experienced cerebral infarctions five times, and he underwent a cerebrovascular anastomosis surgery four years ago. He showed swallow disturbance, general delayed development, hemiplegia, and strabismus. Also he had hypocalcified teeth with or without multiple caries lesions in all dentitions. Dental treatment under general anesthesia using sevoflurane was performed due to his lack of cooperation. Moyamoya disease is associated with various medical conditions requiring a thoughtful deliberation and a careful examination before and during dental treatment. Pain and anxiety control during dental treatment is important because hyperventilation induced by crying has been seen to trigger TIA. Both isoflurane and sevoflurane are commonly used in patients with MMD, but dynamic autoregulation is better preserved during sevoflurane than isoflurane anesthesia. So sevoflurance general anesthesia may be recommendable to manage dental patients having multiple caries with moyamoya disease.

• Journal of Korean Neurosurgical Society
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• v.30 no.6
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• pp.769-773
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• 2001

Moyamoya disease is defined as the development of collateral pathways, associated with bilateral chronic progressive stenosis of the carotid fork. Persistent trigeminal artery is the vessel most frequently observed to persist into adult life among persistent carotid-basilar and carotid-vertebral anastomotic vessels. The authors present a man who had a sudden, severe headache and brain CT showed subarachnoid hemorrhage in left interpeduncular and prepontine cistern. Four-vessel angiogram revealed moyamoya disease associated with aneurysm arising from the junction of persistent trigeminal artery aneurysm and basilar artery. As a treatment, coil embolization was tried but it was failed because of anatomical difficulty of aneurysm. The aneurysm was successfully treated with clipping surgery 10 days later. To our knowledge, this is the first case being reported.

• The Journal of Internal Korean Medicine
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• v.37 no.2
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• pp.420-426
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• 2016

Objective: This case report examined a patient to determine if traditional Korean medicine treatment was effective for treating acute intracerebral hemorrhage (ICH) diagnosed with moyamoya disease.Method: The patient was treated with herbal medicine, acupuncture, and Western medicine and was followed up with for symptoms, vital signs, and brain computed tomography (CT) scans.Results: During the treatment, the patient’s mental state was mostly alert. The patient’s headaches were improved after the use of painkillers; the patient’s fever and blood pressure were well controlled. The cerebral hematoma was removed smoothly.Conclusion: Traditional Korean medical therapy (herbal medicine and acupuncture) along with Western methods appeared to be effective in managing the acute phase of ICH diagnosed with moyamoya disease.

• 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.

• Journal of Korean Neurosurgical Society
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• v.45 no.1
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• pp.50-52
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• 2009

A 31-year-old man presented with dull headache and memory disturbance lasting for one week. Computed tomographic scans revealed acute hydrocephalus. The cerebrospinal fluid contained 53 leukocytes/$mm^3$, with a mononuclear preponderance and no erythrocytes. Magnetic resonance imaging revealed hydrocephalus and leptomeningeal enhancement. Magnetic resonance angiography and digital subtraction angiography showed supraclinoid occlusion of the right internal carotid artery, which resembled unilateral moyamoya disease. Neuroendoscopic biopsy of a lesion in the septum pellucidum revealed noncaseating granulomas, which was consistent with sarcoidosis. The patient was successfully managed with intravenous methylprednisolone and ventriculoperitoneal shunting. To our knowledge, this is the first case of moyamoya-like vasculopathy associated with neurosarcoidosis.

• 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 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.