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

Four adult female patients with moyamoya vessels are described. Hypertension and hyperlipidaemia were discovered in three patients. Vessels similar to moyamoya vessels were seen in the orbital and cortical collateral vessels as well as in the basal ganglia region. Attention should now be paid to the aetiological rather than the descriptive aspects of the disorder. An empirical trial of corticosteroids is worth while. Risk factors such as hypertension, hyperlipidaemia, and smoking should be eliminated.
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PMID:Multiple progressive intracranial arterial occlusions ('moyamoya' disease). 59 61

The authors describe the case of a patient of 22 with hypertension and livedo reticularis who, after presenting with a left brachial monoparesis became progressively demented over a period of five years and died at the age of 27 from a cerebro-meningeal haemorrhage. Angiographically, diffuse distal obliteration of the cerebral arteries was seen with deep networks of the moyamoya type involving the lenticulostriate arteries; similar changes were present in the upper left limb. Histopathological investigations showed obliterative thromboangiitis affecting not only the cerebrum, the brain stem and the cerebellum, but also the viscera. Analysis of this case and consideration of the theoretical possibilities leads the authors to urge that thromboangiosis together with its cerebral manifestations should be considered a nosological entity.
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PMID:[Anatomoclinical and angiographic study of a case of disseminated thromboangiosis with predominant cerebral manifestations]. 98 58

A 59-year-old female patient suddenly developed vomiting and gait disturbances followed by decreasing consciousness. CT scans revealed a hemorrhage within the left basal ganglia region with rupture into the ventricles and consecutive hydrocephalus. On angiography an aneurysm in the region of the caput nuclei caudati was shown to be the source of the bleeding. On repeat-angiography 4 months later the aneurysm was no longer visualized, probably due to thrombosis. This is an extraordinary case of a basal ganglia aneurysm comparable with the aneurysms of Willis' circle, but located in a region where generally microaneurysms--mostly combined with hypertension or moyamoya disease--can be found.
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PMID:Macro-aneurysm in the basal ganglia region. 133 61

Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patients with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.
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PMID:Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography. 149 13

Clinical picture of Moya Moya Disease was analyzed in 18 cases. In 16 cases whose CT scan were available at the time of the first bleeding, hematoma at the basal ganglia was noted in 43.8%, primary ventricular hemorrhage in 37.5%, thalamic hemorrhage with ventricular rupture in 12.5% and subcortical hemorrhage in 6.3%. The frequencies shown above were well correlated to previous reports. In MRI performed 1 year or more after primary ventricular hemorrhage, the primary bleeding site was demonstrated at the lateral wall of the lateral ventricle, in proton 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 5.4 years, 27.8% of the cases had rebleeding one or more times. As a result, good outcome was noted in 72.2% after the 1st bleeding, and in 55.6% after re-bleedings. Death occurred in 5.6% of patients after the 1st bleeding and in 22.2% after further rebleeding. Rebleeding worsened the outcome. Therefore, prevention of rebleeding is important. From a therapeutic viewpoint, although a direct relation between rebleeding and untreated hypertension could not be established, blood pressure control is critical at both the acute and the chronic stages. Reconstructive vascular surgery is a recommendable method for properly selected patients.
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PMID:[Hemorrhagic type of moyamoya disease]. 189 15

Anesthetic management during 85 STA-MCA anastomoses with or without encephalo-myosynangiosis for 64 patients with Moyamoya disease was evaluated retrospectively. Anesthetic agents included nitrous oxide-NLA (GONLA), nitrous oxide-halothane (GOF), nitrous oxide-enflurane (GOE), and their combinations. Slight hypercarbia (40 mmHg less than PaCO2 less than 50 mmHg) was essential to avoid cerebral ischemia. Several procedures to control heart rate by beta blockade or to control hypertension by nitroglycerin were required, because tachycardia and hypertension interfered with fine surgical procedure. During microsurgery HR of GONLA anesthetized patients was significantly lower. Postoperatively the patients anesthetized by GOE showed significantly lower PaCO2 compared with the GONLA anesthetized patients. So we recommend GONLA for anastomosis in patients with Moyamoya disease.
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PMID:[Anesthetic management of revascularization for moyamoya disease]. 192 Jul 89

In order to clarify the cerebrovascular response in Moyamoya disease, the autoregulation and CO2 response was investigated using the 81mKr continuous cerebral blood flow (CBF) measurement technique. A total of 32 measurements were made over the anterior and posterior circulation in 16 Moyamoya patients (seven adults, nine children). CBF measurements were made during four loading trials (hypertension, hypotension, CO2 inhalation and hyperventilation). Study was then made of the vascular response of the frontal lobe, perfused by the internal carotid artery (ICA), and the occipital lobe and cerebellum, perfused by the vertebral artery (VA). Deficits of autoregulation were more severe among the juvenile cases in response to hypotension than to hypertension in both the ICA and VA regions, but the deficits were mild. The CO2 response to hypercapnea in the juvenile cases tended to be abnormal in both the ICA and VA regions. Both adult and juvenile patients showed deficits in the ICA region in response to hyperventilation, some of whom exhibited paradoxial responses. Notable differences in the severity of the deficits of the vascular response in adult and juvenile cases were seen, with the deficits in the response being more severe among the juvenile cases. Moreover, significant regional differences in the deficits were also found.
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PMID:Cerebral blood flow in moyamoya disease. Part 2: Autoregulation and CO2 response. 212 2

We studied five patients with cerebral hemorrhage limited to the head of the caudate nucleus. This rare localization represents 11% of central nuclei hemorrhages. This entity has various clinical expressions; some are similar to a subarachnoid hemorrhage, others show the same manifestations associated with hemiparesis and neuropsychological findings, while still in others, the neuropsychological syndrome with speech, behaviour or memory disturbances predominates. Recovery depends on the patient's previous clinical status and on the presence of associated lesions at the time of hemorrhage. Typical warning signs, like headache, are often absent in the elderly and debilitated. Meningismus is explained by the intraventricular extension of the hemorrhage. Motor deficit, usually moderate, is probably due to impairment of the anterior portion of the posterior arm of the internal capsule. Destruction of the head of the left caudate nucleus, which is part of the circuit causing "subcortical aphasias", is responsible for non specific speech disturbances, that are however remarkably rich in semantic paraphasias. These dysfunctions could be caused by a "cortical diaschisis" as suggested by SPECT analysis. Memory dysfunction as a result of caudate lesion is questioned. However confusion and behavioural disturbances, like preservations, transitory mutism and self neglect, seem characteristic. As shown by cerebral blood flow (CBF) studies, these disturbances might represent a frontal dysfunction caused by the interruption of the dorso-latero-prefrontal and orbito-frontal circuits. When the hemorrhage extends beyond the head of the caudate nucleus, behavioural changes occur due to the involvement of neighbouring structures such as the thalamus, internal capsule, temporal lobe and nucleus accumbens. Caudate hemorrhages occur mostly in the elderly, often with long-standing arterial hypertension causing lesions of the lenticulo-striate arteries. Severe stenosis or complete occlusion of the middle cerebral artery with a fragile anastomotic circuit or angiopathies in younger individuals (particularly Asiatics: moyamoya disease) are less frequent, but they should be considered and investigated by arteriography. Vascular malformations are a rare cause and a relationship with amyloid angiopathy can only be suspected.
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PMID:[Hematoma of the head of the caudate nucleus]. 229 Oct 35

This is a report of a case history of a child with cerebral Moyamoya disease and gradual development of systemic hypertension. Sodium depletion combined with enalapril induced renal failure. A bilateral renal artery stenosis was found. Percutaneous transluminal angioplasty was not successful and was followed by autotransplantation of both kidneys. Histopathological examination of the renal arteries revealed intimal hyperplasia.
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PMID:Moyamoya disease associated with renovascular hypertension. 231 57

We investigated cerebral blood flow and metabolism, and cerebral vascular response in 9 patients with cerebrovascular Moyamoya disease or unilateral Moyamoya phenomenon using positron emission tomography (PET). The subjects consisted of 5 men and 4 women, and were from 9 to 60 years old. Five patients had bilateral occlusion in the carotid fork with Moyamoya vessels (fulfilled the criteria of cerebrovascular Moyamoya disease), and four patients had unilateral Moyamoya phenomenon. The PET scanner used was the HEADTOME III, of which spatial resolution in clinical use was 10 mm full width at half-maximum (FWHM) in the image plane. Cerebral blood flow (CBF), cerebral metabolic rate of oxygen (CMRO2), cerebral oxygen extraction fraction (OEF), and cerebral blood volume (CBV) were measured in resting state by the 15O-labelled gases steady state method in every patient and 22 normal controls (17 men and 5 women, and from 26 to 64 years old). Consecutively cerebral vascular responses were measured by H215O autoradiographic method in resting state, hypercapnia, hypocapnia, and hypertension. Forced hypercapnia, hypocapnia, and hypertension were achieved by 7% CO2 inhalation, hyperventilation, and venous infusion of angiotensin II, respectively. CMRO2 of the whole brain was significantly lower in patients than in normal controls (p less than 0.05), and CBV of the lentiform nucleus significantly increased in patients (p less than 0.01). This reflected Moyamoya vessels in the basal ganglionic regions. In 3 of 5 patients with bilateral Moyamoya vessels, CBF and CMRO2 in the symptomatic cerebral hemisphere were lower than that in the nonsymptomatic hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Vascular responses in cerebrovascular "Moyamoya" disease--evaluated by positron emission tomography]. 251 9


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