• Journal of Chest Surgery
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• v.35 no.3
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• pp.231-234
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• 2002

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries as well as other collateral arteries. However, moyamoya diseases are recently being reported as a systemic process. We experienced one case of coronary artery occlusive disease affected by moyamoya disease. The patient was a 35-year-old female, experiencing intermittent NYHA class ll dyspnea and exertional chest pain for 6 months and right paresthesia for 1 month before admission. Cerebral artery angiogram showed abnormal cerebrovascular systems and confirmed moyamoya disease with cerebral infarction of the left frontal lobe. In coronary artery angiogram, left coronary artery was not visualized due to total occlusion of the left main ostium and left coronary blood flow was supplied from normal right coronary artery. CABG was performed with OPCAB. Both internal mammary arteries were used for LAD and LCx. Intraoperative coronary artery findings showed intimal hyperplasia and no definite thrombi, and nondiseased coronary arteries were good and patent. We concluded that this patient's coronary artery disease was affected by moyamoya disease, and moyamoya disease should be evaluated in the extracerebral cardiovascular system.

• Annals of Clinical Neurophysiology
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• v.18 no.1
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• pp.1-6
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• 2016

Background: Moyamoya disease is characterized by a progressive stenosis or occlusion of the intracranial internal carotid artery and/or the proximal portion of the anterior cerebral artery and middle cerebral artery. Whether the onset time was childhood or adulthood, the bony carotid canal diameter might be different, but reflects the size of internal carotid artery passing through the bony carotid canal. In this study, we aimed to identify the relationship between bony carotid canal diameter and clinical manifestation. Methods: 146 consecutive patients diagnosed with moyamoya disease by brain imaging studies were included. We measured the diameter of a transverse portion of bony carotid canal on bone window of a brain computed tomography(CT) image. Patients were divided into two groups, ischemic or hemorrhagic stroke according to clinical manifestation. As a result, 115 patients were included. The Suzuki stage was used as criteria for disease progression. Results: Bony carotid canal diameter was $3.6{\pm}0.5$ (right) and $3.6{\pm}0.4$ (left) in the hemorrhagic stroke group, and $3.7{\pm}0.4$ (right) and $3.6{\pm}0.4$ (left) in the ischemic stroke group. The bony carotid canal diameter of the moyamoya vessels (3.6 mm) was smaller than the diameter of non-moyamoya vessels (3.8 mm), significantly (p = 0.042). However, there was no difference in the collateral patterns and clinical manifestation in a comparison of both groups. Conclusions: In our study, there was no significant difference of clinical manifestations and collateral patterns depend on the bony carotid canal diameter in patients with moyamoya disease. These findings suggest that the clinical presentations of moyamoya disease are not related to the onset time of the disease.

• 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.

• Clinical and Experimental Pediatrics
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• v.56 no.12
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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.

• Clinical and Experimental Pediatrics
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• v.56 no.12
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• pp.545-549
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• 2013

We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left internal carotid artery and right anterior cerebral artery with accompanying collateral circulation (moyamoya vessels). After the patient underwent bypass surgery on the left side, she recovered from the neurological problems and did not experience any additional ischemic attack during the 14-month follow-up period. This case represents an unusual association between moyamoya syndrome and inactive SLE (inactive for a relatively long interval of 2 years) in a young girl.

• 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 Korean Neurosurgical Society
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• v.41 no.6
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• pp.414-417
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• 2007

A 52-year-old woman with hypertension and moyamoya disease presented with chronic subdural hematoma [CSDH]. The presumed cause of bleeding was ascribed to administrated antiplatelet agents. She responded slowy and clumsily to verbal commands and had right arm weakness. After surgery, her clinical condition improved. But two days after surgery, her symptoms became aggravated and a convulsive seizure was noted within 24 hours. Brain magnetic resonance imaging showed no organic lesion except a small amount of residual CSDH. In addition, there was no laboratory evidence of metabolic brain disease. Moreover, after the administration of an antiepileptic drug [phenytoin], her manifestations disappeared. Therefore, the authors presume that her symptoms were resulted from nonconvulsive status epilepticus [NCSE], despite a lack of ictal period electroencephalographic findings. The authors were unable to find a single case report on postoperative NCSE in Korea. Therefore, the authors report this case of nonconvulsive status epilepticus after drainage of a CSDH in a patient with moyamoya disease.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.772-775
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• 2003

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries, but the etiology remains unclear. Angiographic characteristics include bilateral stenosis or occlusion of the terminal portions of the intracranial internal carotid arteries and bilateral development of fine collateral vessels at the base of the brain known as ‘Moyamoya vessels’. Cardiac surgery using cardiopulmonary bypass due to coronary artery disease and others among patients with moyamoya disease is very rare, and cardiac surgery for such patients has a potential risk of intraoperative and perioperative brain ischemia. We successfully treated a patient who underwent artrial septal defect closure and coronary artery bypass graft using the cardiopulmonary bypass, so we report this case with a brief literature review.

• 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.