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

With 16.9 million people who suffered a first-ever stroke in 2010 worldwide, stroke is a very common vascular disease. Epidemiologic studies have played an essential role in assessing this burden and in detecting the risk factors for stroke. Primary prevention of these risk factors, primarily hypertension, smoking, diabetes, and atrial fibrillation, has reduced the incidence in high-income countries. However, stroke remains a major cause of death and disability, and therefore research should be continued. Subarachnoid hemorrhages are less prevalent than strokes but have an even higher risk of death. Similar to stroke, epidemiologic studies identified smoking and hypertension as its most important risk factors, together with excessive alcohol intake. Although rare, arterial dissections, CADASIL, arteriovenous malformations, venous sinus thrombosis, moyamoya disease, and vasculitis can lead to serious symptoms. The burden and risk factors of those rare diseases are more challenging to assess. Whenever possible, they should be recognized in a timely manner for their increased risk of stroke, but most often they are diagnosed only at the time of stroke. Some cerebrovascular abnormalities do not result in immediate symptoms. This subclinical cerebrovascular disease includes silent infarcts, white-matter lesions, and microbleeds, and is incidentally found by neuroimaging. These lesions are not innocent, as several epidemiologic studies have associated subclinical cerebrovascular disease with an increased risk of stroke, cognitive decline, dementia, and death.
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PMID:Cerebrovascular disease. 2763 62

While intraventricular haemorrhage is frequently found in association with intraparenchymal or subarachnoid haemorrhage, isolated intraventricular haemorrhage (iIVH) is rare in adults and seldom described. Awareness of possible causes is important in order to guide patient management. After elimination of a traumatic cause, numerous aetiologies remain possible. The most frequently found underlying lesions are arteriovenous malformations and aneurysms, but other vascular causes should also be sought, including cavernous malformations and moyamoya disease. Arterial hypertension, anticoagulant use, coagulopathies and certain toxic substances are also associated with iIVH. Finally, iIVH may be caused by intraventricular tumours. In a high number of cases, the cause remains unknown. Vascular and non-vascular causes should be searched through an imaging work-up (with CT angiography, MRI and catheter angiography when necessary) and correlation with clinical information to yield a diagnosis. The aim of this pictorial essay was to review the aetiologies of iIVH in adults.
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PMID:Isolated intraventricular haemorrhage in adults. 2780 21

Studies investigating risk factors for intracranial atherosclerosis (ICAS) have been infrequent. However, due to recent availability of non-invasive vascular imaging techniques that can assess intracranial cerebral arteries, there are a growing number of studies on risk factors for ICAS. Conventional vascular risk factors such as hypertension, diabetes, hypercholesterolemia and cigarette smoking are risk factors for ICAS. However, it remains uncertain whether there is a difference in risk factors between ICAS and extracranial atherosclerosis (ECAS). It also remains unclear why ICAS is more common in Asians and Blacks than in Caucasians. Although we reviewed available evidences on these differences, the review was limited because studies were heterogeneous in the definition of risk factors, diagnostic method, and characteristics of study subjects (hospitalized vs. community) or cerebral vessels (symptomatic vs. asymptomatic). Nevertheless, it seems that hypercholesterolemia is more closely associated with ECAS than ICAS. The difference in hypercholesterolemia prevalence is one of the main reasons for racial differences in the location of cerebral atherosclerosis. Intracranial arteries contain higher antioxidant level than extracranial arteries and may be more vulnerable to risk factors that deplete antioxidants (e.g., metabolic syndrome and diabetes mellitus). Intracranial arteries may be more vulnerable to factors associated with hemodynamic stress (e.g., advanced, salt-retaining hypertension and arterial tortuosity) because of a smaller diameter, thinner media and adventitia, and fewer elastic medial fibers than extracranial arteries. Additionally, non-atherosclerotic arterial diseases (e.g., moyamoya disease) that commonly occur in the intracranial arteries of East Asians may contaminate the reports of ICAS cases. Various genes, including RNF 213, might also explain racial differences in atherosclerotic location. Prospective, well-designed risk factor and genetic studies should be performed in a homogeneous group of patients with diverse ethnicities. These efforts are essential in the prevention of atherosclerotic diseases based on adequate knowledge of the risk factors and pathogenesis.
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PMID:Risk Factors. 2796 Jan 58

OBJECTIVEChoroidal collateral vessels typical of moyamoya disease have received attention as a potential bleeding source. The authors' previous angiographic cross-sectional analysis suggested a possible association between choroidal collaterals and posterior hemorrhage, indicating a high risk for rebleeding. The present longitudinal analysis is intended to determine whether choroidal collaterals are a predictor of rebleeding in hemorrhagic moyamoya disease.METHODSThe Japan Adult Moyamoya Trial group designed an ancillary cohort study using 5-year follow-up data on 37 patients included in the nonsurgical arm of the original randomized controlled trial and compared the rebleeding rate of those with and those without choroidal collaterals, represented by the connection between the anterior or posterior choroidal arteries and the medullary arteries. An expert panel determined whether a choroidal collateral was present in each patient through the measurement of baseline angiography studies. The rebleeding rate comparison was adjusted for age, diagnosis of hypertension, and involvement of the posterior cerebral artery.RESULTSChoroidal collaterals were present in 21 patients (56.8%). The rebleeding rate was 13.1% per year in the collateral-positive group as compared with 1.3% in the negative group (p = 0.008, log-rank test). The adjusted hazard ratio for rebleeding in the collateral-positive group relative to the negative group remained statistically significant (HR 11.10, 95% CI 1.37-89.91). Radiographic assessment of the collateral-positive group revealed good correspondence between the distribution of collaterals and rebleeding sites.CONCLUSIONSResults of this study suggest that choroidal collaterals are a bleeding source with a high risk for hemorrhagic recurrence and a predictor of rebleeding in hemorrhagic moyamoya disease.
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PMID:High rebleeding risk associated with choroidal collateral vessels in hemorrhagic moyamoya disease: analysis of a nonsurgical cohort in the Japan Adult Moyamoya Trial. 2949 73

Severe medical stress can trigger thyroid storm, an endocrine emergency that affects consciousness. This case report describes a 43-year-old female patient transferred to our hospital with right motor hemipare- sis. Her medical history included untreated hyperten- sion, but no history of thyroid disease. Emergency magnetic resonance imaging (MRI)of the head revealed bilateral moyamoya disease and ischemic stroke in the right watershed region of the brain. The right superfi- cial temporal artery was anastomosed to the middle cerebral artery on day 71 of hospitalization due to repeated brain infarctions after admission. Although anesthesia was without incident, the patient gradually developed postoperative disturbed consciousness, hypertension and tachycardia, which we considered were the result of craniotomy or insufficient brain per- fusion, until a nurse found a swelling on the patient's neck. Hyperthyroidism was confirmed by laboratory data and an endocrinologist concluded that thyroid storm had caused her symptoms. Thyroid storm should be considered in the differential diagnosis of patients who present with disturbed consciousness after intracranial surgery.
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PMID:[Thyroid Storm Precipitated by Intracranial Surgery in a Woman with No History of Thyroid Disease.] 3035

A 31-year-old primigravida diagnosed with moyamo- ya disease at age 27, underwent an urgent cesarean section at 37 weeks of gestation. At age 27 the patient had a superficial temporal artery-middle cerebral artery bypass operation, but the blood flow was not enough. Because she had serious anxiety neurosis and could not endure the strain of vaginal delivery or spi- nal anesthesia, general anesthesia was selected. Anesthesia was induced with thiopental, remifentanil and rocuronium, and was maintained with sevoflurane in air oxygen mixture before delivery. After delivery, anesthesia was maintained with propofol and remifent- anil. Hypertension caused by tracheal intubation was successfully prevented by remifentanil. The cesarean delivery was uneventful and a neonate was delivered with Apgar scores of 3 and 9 at 1 and 5 min, respec- tively. No adverse symptoms occurred. Both she and the neonate were discharged without adverse events. Remifentanil was effective for treating perioperative hypertension undergoing caesarean delivery in a patient with moyamoya disease.
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PMID:[General Anesthesia with Remifentanil for Cesarean Section in a Patient with Moyamoya Disease]. 3035 84

The anterior cerebral artery (ACA) is a unique artery with many important variations with substantial clinical significance. Tortuous intracranial arteries usually occur in basilar, communicating, anterior, posterior cerebral arteries and in the white matter arterioles. This could happen for many reasons including but not limited to ageing, hypertension, patients with Moyamoya disease, congenital malformation, or increased flow associated with elastin degradation. While dolichoectasia of the ACA has been described even in children, to our knowledge, a serpiginous ACA without ectasia has not been reported, especially in the pediatric population.
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PMID:A Serpiginous Pericallosal Anterior Cerebral Artery. 3041 May 8

Guo J, Guo L, Zeng G, Tong Z, Gao X, Gu Y. An infant case of renovascular hypertension in moyamoya disease treated by angioplasty. Turk J Pediatr 2018; 60: 331-334. Here we report the treatment experience of an 18-month-old female infant case of renovascular hypertension due to moyamoya disease by angioplasty. Although renal blood flow was recovered immediately after operation, 12 months follow-up found severe restenosis which required us to perform a right renal autotransplantation.
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PMID:An infant case of renovascular hypertension in moyamoya disease treated by angioplasty. 3051 50

Moyamoya disease is a rare condition affecting the circle of Willis and its branching arteries. While the pathogenesis is unclear, it causes progressive occlusion of multiple cerebral vessels leading to severe strokes. We report a case of a 47-year-old Hispanic woman with HTN presented with altered mental status and bilateral upper and lower extremity weakness with dystonic-like upper extremity movement. Serial brain CTs and angiography were performed which showed massive frontal and parietal cerebral infarcts with radiological evidence of moyamoya disease.
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PMID:Bilateral Cerebrovascular Stroke as an Initial Presenting Symptom of Moyamoya Disease. 3053 67

Background: Alagille syndrome (AGS) is an autosomal-dominant, multisystem disorder caused by mutations in the JAG1 gene. Case Description: A 34-year-old man was referred to our service 10 years ago with focal seizures with impaired awareness and transient slurred speech. He had a 5-year history of intermittent left monocular low-flow retinopathy. He has a family history of AGS. General examination revealed mild hypertension, aortic regurgitation, and livedo reticularis. Neurological examination was normal. Investigations: He had mild hyperlipidaemia and persistently-positive lupus anticoagulant consistent with primary anti-phospholipid syndrome. Color Doppler ultrasound revealed low velocity flow in a narrowed extracranial left internal carotid artery (ICA). MR and CT angiography revealed a diffusely narrowed extracranial and intracranial left ICA. Formal cerebral angiography confirmed severe left ICA narrowing consistent with a left ICA "vasculopathy" and moyamoya phenomenon. Transthoracic echocardiogram revealed a bicuspid aortic valve and aortic incompetence. Molecular genetic analysis identified a missense mutation (A211P) in exon 4 of the JAG1 gene, consistent with AGS. Discussion: AGS should be considered in young adults with TIAs/stroke and unexplained extracranial or intracranial vascular abnormalities, and/or moyamoya phenomenon, even in the absence of other typical phenotypic features. Gene panels should include JAG1 gene testing in similar patients.
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PMID:Extracranial and Intracranial Vasculopathy With "Moyamoya Phenomenon" in Association With Alagille Syndrome. 3076 Oct 79


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