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Query: UMLS:C0026654 (moyamoya)
 2,046 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of well-known syndrome due to midbrain-brain stem hemorrhage without having a history of hypertension were reported. Case 1 is a 62-year-old female, presenting Weber's syndrome due to midbrain hemorrhage and diagnosed as Moyamoya disease angiographically. Case 2 is a 24-year-old-male, presenting One and a half syndrome due to brain stem hemorrhage from a brain stem cavernous angioma, which was diagnosed angiographically and by magnetic resonance imaging (MRI). Left hemiparesis seen in case 1 had improved by conservative therapy but no improvement could be obtained in the oculomotor nerve palsy. In case 2, hematoma was aspirated surgically for the purpose of decompression and his neurological symptoms improved markedly. Their pathogenesis and pathophysiology were also discussed.
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PMID:[Two cases of well-known syndrome due to midbrain-brain stem hemorrhage--Weber's syndrome and one and a half syndrome]. 847 67

The nucleus caudatus is not a common location of spontaneous intracerebral hemorrhage. Twelve patients (8 men and 4 women) aged 38-76 years who had caudate hemorrhage between November 1, 1992 and March 31, 1994 were evaluated. These cases represented 2.1% of intracerebral hemorrhage cases at Neurological Institute, Veterans General Hospital-Taipei, Taiwan. Cerebral angiography was performed on eight patients. Six patients were evaluated by an extensive neuropsychological battery. The most frequent symptoms of caudate hemorrhage were headache and/or vomiting, and decreased consciousness. Clinical features were similar to those of subarachnoid hemorrhage. Angiography showed characteristic moyamoya disease in one patient but did not show an aneurysm or arteriovenous malformation in any patient. The etiology in most patients was hypertension. Neuropsychological assessments showed significant impairment in tasks of short-term and long-term memory and in verbal fluency as well as trends of impairment of orientation rather than of controls. Neurobehavioral symptoms probably resulted from interruption of the cortical-subcortical loops between the caudate nucleus and prefrontal cortex.
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PMID:Caudate hemorrhage: clinical features, neuropsychological assessments and radiological findings. 859 95

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

Over the past 28 years, 39 patients with Moyamoya disease or syndrome defined as spontaneous occlusion of the circle of Willis with extensive basal collateral vessels have been treated by the author in Canada and the USA. All patients presented with clinical or radiologic evidence of hemorrhage (23) or ischemia and infarction (16). A total of 12 patients had associated cerebral aneurysms and seven of these patients with aneurysms presented with subarachnoid hemorrhage. The patients ages ranged from 5 to 47 years. Of these 58% were female. The patients racial origin included North American Indian, Innuit, East Indian/Pakistani, Japanese, Chinese, Filipino, Korean, Malayasian, Hispanic, African American and Caucasian. Familial clustering was seen in North American Indian, Innuit and Caucasian patients. Associated disorders (tuberculosis, pharyngitis, thalassemia, fibromuscular hyperplasia, polycystic kidney, sickle cell trait and hypertension) were common in these patients, as was the use of tobacco, alcohol and in the adult females, oral contraceptives. It may be concluded from this series that the etiology of Moyamoya disease or syndrome is probably multifactorial, but that some racial and familial groups are more susceptible. Furthermore, in that the clinical and angiographic features are identical, the separation between Moyamoya disease and syndrome may not be helpful in understanding the etiology and pathophysiology of this disorder.
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PMID:Risk factors of moyamoya disease in Canada and the USA. 940 4

The clinical picture of hemorrhagic type Moyamoya disease was analyzed in 20 cases. Hematoma at the basal ganglia was noted in 40% of cases, intraventricular hemorrhage (IVH) in 30%, thalamic hemorrhage with ventricular rupture in 15% and subcortical hemorrhage in 5%. The location was undetermined in two cases (10%) due to bleeding in the pre-computed tomography (CT) era. The frequencies shown above were correlated well to previous reports. In magnetic resonance imaging (MRI) performed 1 year or more after IVH, the primary bleeding site was demonstrated at the lateral wall of lateral ventricle, in portion density weighted and T2 weighted images. MRI can detect the site of old bleeding points and its chronological change if the study is repeated. In a follow-up period of 6.2 years, 35% of the cases had rebleeding once or twice. The second bleeding occurred seven times and the third twice. IVH occurred five times and the most common, basal ganglia hematoma three times while thalamic hemorrhage once. As a result, the rate of good outcome was 60% after the first bleeding and 40% after rebleeding. The mortality rate was 5% after the first 25% after rebleedings. Factors related to rebleedings and their poorer outcome are sex (with women being more susceptible), massive ICH and early recurrence. Rebleeding worsened the outcome. Therefore, prevention of rebleeding is important. From a therapeutic viewpoint, although a close relation between rebleeding and untreated hypertension could not be established, blood pressure control is critical at the both acute and chronic stages. Bypass surgery for bleeding type of Moyamoya disease seems to be less promising than ischemic type, even though a definite answer may not be obtained from our small number of cases.
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PMID:Hemorrhagic type moyamoya disease. 940 37

Moyamoya disease is an occlusive intracranial arteriopathy with abnormal cerebral vascular collateral networks. Although this disease is known to be associated with stenosis of extracranial arteries, such as the renal artery, a case associated with stenoses of the coronary and renal arteries has not been reported. We described here a 23-year-old man who had effort angina, renovascular hypertension, and asymptomatic moyamoya disease. Arterioangiography revealed bilateral occlusion of the intracranial carotid arteries and stenoses in the left coronary artery and the left renal artery.
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PMID:Asymptomatic moyamoya disease associated with coronary and renal artery stenoses--a case report. 955 34

Neurofibromatosis Type 1 produces a broad spectrum of clinical manifestations as a result of widespread dysplasia of mesodermal and neuroectodermal tissues. One of the most serious aspects of the disease relates to the arterial involvement that may occur. We report a case of severe stenosis of the internal carotid artery and intracerebral hemorrhage associated with neurofibromatosis Type 1. A 49-year-old female was admitted to our hospital after she had suddenly become comatose. On admission, she demonstrated a decerebrate posture in response to painful stimuli, and was assessed as grade 200 according to the Japan Coma Scale. Physical examination disclosed widespread cutaneous neurofibromas and cafe-au-lait spots. Computed tomography of the head revealed a right putaminal extensive hematoma, with a maximum diameter of 7 cm. The hematoma was removed. After this surgical treatment, cerebral angiography was performed. It showed severe stenosis of the terminal portion of the right internal carotid artery associated with a fine telangiectatic network, indicating the presence of moyamoya vessels in the basal ganglia. Although intracranial hemorrhage associated with neurofibromatosis type 1 is a rare condition, fine telangiectatic collateral vessels caused by occlusive cerebrovascular disease, intracranial aneurysms, brain tumors, or hypertension caused by pheochromocytoma or stenosis of the renal artery should be considered as the cause of hemorrhage.
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PMID:[Severe stenosis of the internal carotid artery and intracerebral hematoma associated with neurofibromatosis type 1: a case report]. 1002 86

From January 1992 to December 1997, 13 parturients with cerebrovascular diseases had childbirth at our institution. Among them, 8 patients received anesthesia for delivery. Five patients had a history of ruptured arteriovenous malformation (AVM), cerebral aneurysm, or intraventricular bleeding due to moyamoya disease, and they had radical operations. Of these 5 patients after radical operations, three had a repeat cesarean section under spinal anesthesia, and two had a vaginal delivery under epidural anesthesia to avoid excessive hypertension and hyperventilation. There were two patients with a history of cerebrovascular diseases but had no radical operations. Of these two, one patient who was diagnosed as having aneurysm underwent elective cesarean section under spinal anesthesia, and another patient with a history of cerebral bleeding underwent cesarean section under general anesthesia for abruptio placentae. These 7 patients did well during pregnancy and puerperium. The eighth patient experienced severe headache followed by loss of consciousness caused by ruptured AVM, and required an emergency operation. Simultaneous cesarean section and craniotomy were performed at another hospital. Intrauterine fetal death (IUFD) occurred, but mother survived.
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PMID:[Anesthetic management of parturients with cerebrovascular diseases]. 1043 20

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

We had performed indirect revascularization surgery, mainly EMS, for cases with moyamoya disease, because EMS can revascularize a large area including the territory of anterior cerebral artery. However, in our initial cases, we found that three sides in two cases had post-operative ischemic complications. These cases suggested that intracranial hypertension due to pressure exerted by swelling caused by edema in the myoflap after EMS was one of the reasons for these infarctions. For the prevention of intracranial hypertension due to the edema in the myoflap, when we did EDAMS with dural pedicle insertion, we put into practice the new ideas about shaving the boneflaps to half of their original thickness, and prescribed 20% Mannitol after surgery. We tried these new ideas concerning treatment on four sides in three cases with moyamoya disease, and we were able to get good outcomes without any new neurological deficits.
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PMID:[New ideas for indirect revascularization surgery for moyamoya disease]. 1056 41


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