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13 patients under 40 years of age had cerebral infarction and angiographic evidence of arterial stenosis or occlusion. None of them had cardiac disorders prone to cerebral embolism. Five patients had occlusion at the origin of the internal carotid artery, one had occlusion of the common carotid artery and one each had stenosis at the origin of the internal carotid and common carotid arteries, respectively. Two patients had unilateral occlusion of the supraclinoid portion of the carotid artery with basal collaterals that had some resemblance to the Moyamoya disease. Three patients demonstrated stenosis or occlusion of the middle and/or anterior cerebral arteries. Three patients had hypercholesterolemia, one of whom was hypertensive. None had confirmed diabetes mellitus. One female, who died, had taken oral contraceptives for 3 years. A male, with internal carotid artery occlusion, had serological evidence for syphilis.
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PMID:Occlusive cerebrovascular disease in young adults. 61 2

A 34-year-old man with hypertension and diabetes mellitus developed dizziness and visited our institute. He had history of headache with numbness of the right hand since age 15 years and left occipital lobe infarction at age 28 years. The cerebral angiogram showed several changes peculiar to advanced stage of moyamoya disease (spontaneous occlusion of the circle of Willis), i.e. segmental stenoses or occlusions of bilateral internal carotid arteries, left vertebral artery and left posterior cerebral artery with abnormal vascular networks at the bilateral basal ganglia. He was also diagnosed to have asymptomatic ischemic heart disease. The coronary angiogram showed diffuse sclerotic lesions of left anterior descending and right coronary arteries without significant stenosis, which suggested the presence of microvascular lesion as a cause of myocardial ischemia. Coronary disease has been rarely reported as a complication of moyamoya disease, and microvascular coronary artery disease has never been described. Moyamoya disease should be regarded as a part of systemic vascular disorders, and the evaluation of extracerebral cardiovascular system is necessary to clarify pathophysiology of this disease.
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PMID:[Systemic vascular change associated with moyamoya-like cerebrovascular disease and microvascular coronary artery disease]. 875 87

We report a case of Prader-Willi syndrome (PWS) complicated with juvenile stroke. The patient is a 19-year-old man with right hemiplegia, who has had a history of non-insulin-dependent diabetes mellitus (NIDDM) for ten years. The diagnosis of PWS was confirmed genetically by the method of fluorescence in situ hybridization which showed the deletion of chromosome 15. His brain MRI revealed abnormal signal intensities in the left basal ganglia and around the right trigone of the lateral ventricle. Angiographic examination showed occlusions of bilateral proximal middle cerebral arteries with basal moyamoya vessels. The left vertebral artery was also occluded at its origin. Only a few cases of PWS complicated with stroke have been reported before and, to date, there has been no case with arterial occlusion similar to our case. Though the cause of these arterial occlusions is unknown, it may be related to arteriosclerosis following NIDDM.
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PMID:[A case of Prader-Willi syndrome with bilateral middle cerebral artery occlusion and moyamoya phenomenon]. 893 99

An 18 year-old woman received radiation therapy for hypothalamic astrocytoma at the age of 11 years. She developed progressive cerebral occlusive vascular disease with moyamoya vessels formation in both carotid systems. Apart from diabetes mellitus, she had no other risk factors for occlusive cerebrovascular disease. The site of occlusion was confined to the field of radiation and the development of moyamoya vessels strongly suggestive of a radiation-induced cause. Radiation therapy around the sella and parasellar region appears to be the most common risk factor for this vasculopathy. Progressive irradiation-induced cerebral vasculopathy is due to accelerated atherosclerosis.
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PMID:Progressive cerebral occlusive disease after hypothalamic astrocytoma radiation therapy. 917 79

A 65-year-old man who had a history of diabetes mellitus visited a hospital for recent memory disturbance. MR angiography showed stenoses of the main trunks of the intracranial arteries. He was admitted to our hospital, and angiography revealed occlusion of the left cervical internal carotid and right middle cerebral arteries, and stenoses of the right internal carotid, right anterior cerebral, left vertebral, and right posterior cerebral arteries. There was collateral circulation formed from the left external carotid and left posterior cerebral arteries. Basal moyamoya vessels were also observed. 123I-IMP SPECT showed low perfusion of the right cerebral hemisphere, and response to acetazolamide was poor. Based on the angiographical findings and the presence of diabetes mellitus, the patient was diagnosed as having quasi-moyamoya disease. Vascular reconstructive surgery was indicated. Since there was no recipient artery adequate for STA-MCA anastomosis, encephalo-duro-arterio-synangiosis was performed on the right side. Postoperatively, the patient's recent memory improved gradually, and angiography performed 7 months after the operation revealed neovascularization from the superficial and deep temporal arteries. Although indirect bypass surgery has been applied mainly to child moyamoya disease, its effectiveness for cerebral ischemia in adult patients has also been reported, particularly in cases with misery perfusion. Neovascularization with indirect bypass surgery can be expected if the patient is under misery perfusion, even in elderly patients.
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PMID:[Neovascularization after encephalo-duro-arterio-synangiosis in elderly quasi-moyamoya disease: a case report]. 930 Apr 48

The efficacy of encephalo-myo-synangiosis (EMS) using muscle free flap was evaluated for the treatment of ischemic cerebrovascular disease in adult patients. Three patients with adult moyamoya disease and three patients with atherosclerotic ischemic cerebrovascular disease were treated. EMS used four latissimus dorsi muscles and two serratus anterior muscles. Postoperative selective angiography showed collateral circulation from the transferred muscle to the brain in four of the six patients. The other two patients showed patent nutrient artery of the transferred muscle flap. Cerebral blood flow study disclosed postoperative improvement of perfusion reserve capacity in all sides. One patient suffered a perioperative stroke by hemoconcentration due to poor control of diabetes mellitus. The mean follow-up period was 23 months. EMS using muscle free flap is a possible procedure in selected patients with impaired cerebral perfusion reserve capacity due to multiple stenosis or occlusion of cerebral arteries including moyamoya disease or who required cerebral blood flow augmentation in the anterior and/or posterior cerebral artery territories due to internal carotid artery occlusion.
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PMID:Cerebral revascularization using muscle free flap for ischemic cerebrovascular disease in adult patients. 978 Jun 43

We report a series of American adults with idiopathic steno-occlusive disease of the supraclinoid internal carotid artery and its bifurcation. We reviewed the clinical records and imaging of 18 patients, 16 women and 2 men, aged 20-53 years (mean 35 years). There were no predominating risk factors for vascular occlusive disease, such as oral contraceptive use, hypertension, diabetes mellitus, or smoking. Four patients had irregularity of their cervical internal carotid arteries in a pattern not classic but suspicious of fibromuscular dysplasia. Eleven patients met the criteria for moyamoya disease, having both bilateral disease and moyamoya collateral lenticulostriate arteries. Necropsy in one case showed intimal thickening with duplication of the internal elastic lamina involving the internal carotid artery bifurcation bilaterally. We found a marked predominance of young and middle-aged females in our American adults, but our findings do not support the association with birth-control pills previously reported.
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PMID:Idiopathic supraclinoid and internal carotid bifurcation steno-occlusive disease in young American adults. 1055 29

Asymptomatic small hemorrhages were identified in hypertensive patients by T2*-weighted gradient echo magnetic resonance (MR) imaging to investigate the relationship between hypertensive intracerebral hemorrhage and asymptomatic minute hemorrhages. Forty-eight patients with hypertensive intracerebral hemorrhage or cerebral infarction with hypertension (these diseases were defined as stroke) were treated in National Defense Medical College from April 1998 to February 2000. All patients had no past history of stroke or head injury, underwent MR imaging within 6 months of the stroke attack, were aged from 40 to 80 years, and had no diagnosis of aneurysm, angioma, or moyamoya disease. Patients were divided into the infarction group and hemorrhage group. All foci over 2 mm in size appearing as hypointense on T2*-weighted MR imaging and unrelated to stroke areas were defined as minute hemorrhages. There were no significant differences between the two groups with respect to sex, age, and history of diabetes mellitus. The incidence of minute hemorrhages in the hemorrhage group (21/26) was greater than in the infarction group (9/22, p < 0.01). The incidence of minute hemorrhages in the basal ganglia (18/26) was greater in the hemorrhage group than in the infarction group (4/22, p < 0.001). Symptomatic intracerebral hemorrhage may be preceded by asymptomatic minute hemorrhage.
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PMID:Relationship between stroke and asymptomatic minute hemorrhages in hypertensive patients. 1121 34

We here report one case of hemichorea and Broca aphasia occurred with diabetic ketoacidosis. A 20-year-old woman with type 1 diabetes mellitus had experienced diabetic ketoacidosis fourth time after the onset of diabetes. At the third ketoacidotic episode, the patient was admitted to our hospital for the first time to show hemichorea of the left extremities. Brain computed tomography (CT) demonstrated a high-density area in the right caudate head and low-density area in the right putamen. Magnetic resonance angiography (MRA) demonstrated a stenosis at the root of the bilateral middle and anterior cerebral arteries. The hemichorea disappeared within 3 days. At the fourth ketoacidotic episode, not hemichorea but unconsciousness was there for 2 days even after ketoacidosis disappeared. After the unconscious state, Broca aphasia was demonstrated for 15 days. The cerebral angiography showed a finding compatible to Moyamoya disease. These findings support that chorea and Broca aphasia induced by diabetic ketoacidosis was developed in addition to blood vessel abnormalities such as Moyamoya disease. We suggest that poorly controlled diabetic patients with hemichorea should undergo cerebral angiography.
Diabetes Res Clin Pract 2005 Feb
PMID:Chorea and Broca aphasia induced by diabetic ketoacidosis in a type 1 diabetic patient diagnosed as Moyamoya disease. 1564 79

A 12-yr-old Kosovo-Albanian boy with insufficiently controlled type 1 diabetes since his second year of life developed severely increased intima-media thickness (IMT) and roughness (IMR) of the common carotid artery (CCA): max/mean IMT=0.81/0.68 mm and IMR=0.048 mm. Intima-media thickening, comparable with that in a 50- to 60-yr-old healthy adult, decreased within 41 months (max/mean carotid IMT=0.72/0.56 mm and IMR=0.036 mm) by intensive treatment of diabetes. Moyamoya disease (MMD), complicated by cerebral infarction, occurred coincidentally but regressed within 6 months. This case report points out that (i) chronic hyperglycemia in childhood may lead to adult-like increase of carotid IMT/IMR as early signs of subclinical atherosclerosis, (ii) increased carotid IMT/IMR may be regressive by intensive diabetes control, and (iii) a screening examination for carotid IMT/IMR should be considered in patients at high risk of atherosclerosis.
Pediatr Diabetes 2005 Sep
PMID:Adult-like but regressive increase of intima-media thickness and roughness in a child with type 1 diabetes. 1610 73


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