Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026654 (moyamoya)
 2,046 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya ("puff of smoke") syndrome is a disease of children and young adults caused by fibromuscular dysplasia of the internal carotid and proximal cerebral arteries and is rarely associated with hypertension. We report a 34-year old woman with moyamoya syndrome who presented with symptomatic severe hypertension associated with unilateral renal artery stenosis. The patient underwent percutaneous transluminal angioplasty of the right renal artery with subsequent improvement in blood pressure control on reduced, and later, no antihypertensive therapy. This case illustrates that moyamoya syndrome may be an intracranial manifestation of a systemic arterial disorder. Renal artery stenosis may, in appropriate cases, be managed by percutaneous transluminal angioplasty.
 ...
PMID:Renovascular hypertension in moyamoya syndrome. Therapeutic response to percutaneous transluminal angioplasty. 297 77

The criteria of the cerebrovascular Moyamoya disease is defined by the characteristic findings of its cerebral angiograms, as follows; 1) The internal carotid siphon is narrowed or obstructed bilaterally. 2) The "Moyamoya vessels" are observed at the base of the brain or the basal ganglionic regions. 3) Main trunks of the cerebral arteries such as the anterior, the middle, and/or the posterior cerebral arteries are often not or poorly visualized. 4) Its etiology is unknown. It has been known that the occlusion of the internal carotid fork with Moyamoya vessels is not infrequently seen in patients with tuberculous meningitis, sickle cell anemia, head trauma, and so on. In the definition of the disease, patients with known etiology and/or unilateral occlusion in the carotid fork must be excluded. However, the cases who cannot fulfil its criteria of the cerebrovascular Moyamoya disease, but have its characteristic Moyamoya vessels and collateral pathways have been reported. We investigated the findings of cerebral computed tomograms in 13 patients who did not fulfil the criteria of the cerebrovascular Moyamoya disease, but revealed the Moyamoya vessels. The subjects are 5 males and 8 females, ranging 15 to 70 years old. The past histories of 9 patients among them revealed hypertension, radiation therapy for pituitary adenoma, head trauma, aplastic anemia, and the Raynaud phenomenon. By angiographic evaluations, occlusions in the unilateral carotid forks were seen in 7 patients, and stenoses in those were in 5 patients. One patient showed only a severe stenosis in the horizontal portion of the middle cerebral artery.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Cases similar to cerebrovascular moyamoya disease--investigation by angiography and computed tomography]. 319 93

Because of the advance of techniques and age-matched apparatus for blood pressure measurement, and because of the availability of age-related normal values in childhood, the knowledge of the number of children having elevated blood pressures has recently improved. In a group of healthy children, the first important task is to determine how many incidences of "essential hypertension" there are among them, which may appear in childhood and persist into adulthood. This should urge us to undertake periodical examinations of healthy children. On the other hand, the treatment of "secondary hypertension" has similarly been improved. Since 1979 in particular, captopril, an orally active angiotensin I-converting enzyme inhibitor, has successfully been administered to treat children with malignant hypertension and who respond poorly to conventional antihypertensive therapies. We report 3 cases that received captopril for refractory hypertension: a 2-year-old boy with renal and renovascular anomalies, a 7-year-old boy with moyamoya disease after surgical operation, and a 17-year-old youth with Cushingoid syndrome due to chronic administration of steroids against mixed connective tissue disease. After the introduction of captopril, good pressure control was obtained in all 3 cases, although reasonable effects of measurement values of the renin-angiotensin-aldosterone system (decrease in angiotensin I & II, increase in I/II ratio, etc.) were found only in the first case.
 ...
PMID:Refractory hypertension in childhood--efficacy of captopril therapy. 332 1

The prognosis of 27 patients with moyamoya disease was studied. The ages at onset ranged from 11 months to 4-11/12 years. Follow-up study was performed within 4 years from the onset in 13, 5 to 9 years in 5, and 10 to 15 years in 9. Transient ischemic attacks (TIA) occurred most often during the first four years and decreased thereafter. Intellectual deterioration and neurologic deficits increased with time. Outcome included no sequelae in five (19%), occasional TIA or headache alone in nine (33%), mild intellectual and/or motor impairment in seven (26%), requirement for special school or care by parents or institutions after reaching the teen years in three (11%), continuous 24-hour care in two (7%), and death in one (3%). Poor prognosis was correlated with an early age at onset and hypertension.
 ...
PMID:Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children. 388 Apr 14

A 29 year-old pregnant woman with occlusive disease of the internal carotid arteries and formation of collateral networks (moyamoya disease) is reported. Because of moyamoya disease and toxemia of pregnancy, cesarean section was performed at the 38th week of pregnancy. The patient had a second intracranial hemorrhage 7 months after cesarean section. Encephalo-duro-arterio synangiosis (EDAS) was performed for the prevention of further intracranial hemorrhage and at present she is well. Since intracranial hemorrhage is sometimes associated with bearing down and since hyperventilation-induced cerebral ischemia and hypertension are provoked by active labor, elective cesarean section may be recommended for pregnant women with moyamoya disease.
 ...
PMID:Occlusive disease of the internal carotid arteries with vascular collaterals (moyamoya disease) in pregnancy. 395 53

Moyamoya is an intriguing and controversial syndrome. This patient study serves to align the pathophysiology of intracranial hemorrhage in moyamoya with the cerebral vascular disease seen with hypertension, or aging. The historical evidence linking lipohyalinosis and microaneurysms to cerebral hemorrhage is reviewed, the pathogenesis of this angiopathy is discussed, and explanations considered for its association with the vascular pattern of moyamoya. We propose that hemodynamics, genetics or both are among the primary operant etiologic factors.
 ...
PMID:Lipohyalinosis and miliary microaneurysms causing cerebral hemorrhage in a patient with moyamoya. A clinicopathological study. 615 50

Findings at the autopsy of a 7-year-old Japanese girl with moyamoya disease associated with renovascular hypertension are reported. There was disruption of a saccular aneurysm located at the junction of the left posterior cerebral and the posterior communicating arteries. Intimal thickening of carotid, coronary, and renal arteries, similar to findings in cases of intimal hyperplasia of fibromuscular dysplasia, was observed. These findings indicate that moyamoya disease may actually be an intracranial manifestation in a systemic arterial disorder.
 ...
PMID:Moyamoya disease associated with renovascular hypertension. 669 36

A unique case is described of subarachnoid hemorrhage from a ruptured berry aneurysm of the right anterior ethmoidal artery, and its pathogenesis is discussed. The literature suggests an increased incidence of posterior circulation aneurysms in moyamoya disease, of contralateral anterior circulation aneurysms in agenesis of the carotid artery, and of aneurysms at various sites in spontaneous and iatrogenically acquired obstructions of the internal carotid artery. In addition to congenital anomalies of the vessel wall and systemic hypertension, increased regional blood flow should be regarded as an important factor in the generation of berry aneurysms.
 ...
PMID:Ruptured berry aneurysm of the anterior ethmoidal artery associated with bilateral spontaneous internal carotid artery occlusion in the neck. Case report. 688 91

Moyamoya disease is a rare disorder characterized by progressive occlusive cerebral arteriopathy associated with an extensive network of collateral vessels. Renovascular hypertension has rarely been associated with this disease. We report on a girl with renovascular hypertension and 2 renal artery aneurysms who was found to have moyamoya disease. Successful surgical treatment was achieved with extracorporeal arterial repair and renal autotransplantation. The relationship between moyamoya disease and renovascular hypertension is reviewed.
 ...
PMID:Surgical treatment of renovascular hypertension in moyamoya disease: case report and review of the literature. 781 17

Extracranial manifestations of vascular occlusive disease, such as renovascular hypertension, are rare in moyamoya syndrome. Histopathological examination suggests a common denominator. Surgical or endoluminal correction of these lesions is feasible. Moyamoya syndrome is not considered to be a contraindication for organ donation for transplantation.
 ...
PMID:Renal artery stenosis in moyamoya syndrome. 799 38


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>