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Neurologia Medico-chirurgica Jul 2020Some of the pediatric moyamoya patients spend their childhood without diagnosed as moyamoya disease (MMD) because of their mild ischemic attacks and emerge again with...
Some of the pediatric moyamoya patients spend their childhood without diagnosed as moyamoya disease (MMD) because of their mild ischemic attacks and emerge again with ischemic or hemorrhagic stroke in their adulthood. This study was aimed to clarify the clinical characteristics of adult moyamoya patients with childhood onset and elucidate the impact of long disease period on their clinical features. Present study included 116 untreated hemispheres of 69 adult patients with MMD. They were divided into two groups: childhood onset group (26 hemispheres of 14 patients) and adult onset group (90 hemispheres of 55 patients). Clinical features were compared between the two groups. The incidence of hemorrhagic stroke was significantly higher in childhood onset group (P = 0.0091). Lenticulostriate and choroidal channels were more developed in childhood onset group (P = 0.044 and P <0.001, respectively). Vault moyamoya was more frequently observed in childhood onset group (P <0.001). The development of surgical collaterals through indirect bypass was more marked in childhood onset group (P = 0.0019). Multivariate analysis revealed that childhood onset and developed choroidal channels were significantly associated with the occurrence of hemorrhagic stroke (OR 4.31 [95% CI 1.21-15.4], P = 0.025 and OR 6.78 [95% CI 1.78-25.8], P = 0.0050, respectively). This study clearly shows that adult moyamoya patients with childhood onset have more developed spontaneous collaterals, which may, in turn, highly causes hemorrhagic stroke. Adult moyamoya patients with "childhood onset" should be recognized as a novel and important concept when elucidating the underlying mechanisms of hemorrhagic stroke in MMD.
Topics: Adult; Age of Onset; Child; Cohort Studies; Female; Hemorrhagic Stroke; Humans; Male; Middle Aged; Moyamoya Disease; Prevalence; Radiography
PubMed: 32536657
DOI: 10.2176/nmc.oa.2020-0001 -
A Review of Artificial Intelligence in Cerebrovascular Disease Imaging: Applications and Challenges.Current Neuropharmacology 2022A variety of emerging medical imaging technologies based on artificial intelligence have been widely applied in many diseases, but they are still limitedly used in the... (Review)
Review
BACKGROUND
A variety of emerging medical imaging technologies based on artificial intelligence have been widely applied in many diseases, but they are still limitedly used in the cerebrovascular field even though the diseases can lead to catastrophic consequences.
OBJECTIVE
This work aims to discuss the current challenges and future directions of artificial intelligence technology in cerebrovascular diseases through reviewing the existing literature related to applications in terms of computer-aided detection, prediction and treatment of cerebrovascular diseases.
METHODS
Based on artificial intelligence applications in four representative cerebrovascular diseases including intracranial aneurysm, arteriovenous malformation, arteriosclerosis and moyamoya disease, this paper systematically reviews studies published between 2006 and 2021 in five databases: National Center for Biotechnology Information, Elsevier Science Direct, IEEE Xplore Digital Library, Web of Science and Springer Link. And three refinement steps were further conducted after identifying relevant literature from these databases.
RESULTS
For the popular research topic, most of the included publications involved computer-aided detection and prediction of aneurysms, while studies about arteriovenous malformation, arteriosclerosis and moyamoya disease showed an upward trend in recent years. Both conventional machine learning and deep learning algorithms were utilized in these publications, but machine learning techniques accounted for a larger proportion.
CONCLUSION
Algorithms related to artificial intelligence, especially deep learning, are promising tools for medical imaging analysis and will enhance the performance of computer-aided detection, prediction and treatment of cerebrovascular diseases.
Topics: Arteriovenous Malformations; Artificial Intelligence; Humans; Machine Learning; Moyamoya Disease
PubMed: 34749621
DOI: 10.2174/1570159X19666211108141446 -
PloS One 2023Disease-specific interventions for management and health behavior implementation are needed to improve the health and quality of life of adolescents with moyamoya... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Disease-specific interventions for management and health behavior implementation are needed to improve the health and quality of life of adolescents with moyamoya disease.
OBJECTIVE
This study aimed to develop a program for adolescents with moyamoya disease based on the salutogenesis theory, which focuses on the process of enhancing health through successful adaptation to external stressors, and to evaluate its effectiveness.
METHODS
A randomized controlled trial was performed according to the CONSORT guidelines. This preliminary research and experimental treatment were conducted at a Severance Hospital ward and outpatient clinic among 48 participants randomized into the intervention (seven sessions of salutogenesis program, n = 24) or the control group (one session of one-to-one moyamoya disease education program, n = 24) from September 6, 2018 to January 4, 2019. Changes in the following study outcomes were reported: "knowledge of moyamoya disease," "social support," "sense of coherence," "moyamoya disease health behavior," "stress," "depression," "subjective health status," "frequency of ischemic symptoms," and "quality of life".
RESULTS
The salutogenesis program improved the knowledge and social support of adolescents with illness-related problems and helped them attain healthy behaviors and stress reduction. It was confirmed to be effective in improving their quality of life.
CONCLUSIONS
The salutogenesis program for adolescents with moyamoya disease effectively improved the generalized resistance resources and sense of coherence in adolescents with moyamoya disease.
TRIAL REGISTRATION
Korean Clinical Research Information Service registry, KCT0006869.
Topics: Humans; Adolescent; Sense of Coherence; Quality of Life; Moyamoya Disease; Health Behavior
PubMed: 37883389
DOI: 10.1371/journal.pone.0284015 -
Neurologia Medico-chirurgica 2012Research on moyamoya disease has progressed remarkably in the past several decades. Indeed, many new facts concerning the epidemiology of the disease have been revealed... (Review)
Review
Research on moyamoya disease has progressed remarkably in the past several decades. Indeed, many new facts concerning the epidemiology of the disease have been revealed and surgical treatments have been drastically improved. However, despite extensive research, the mechanism of moyamoya disease is still unknown. Consequently, the cardinal treatment of this disease has not yet been developed. For further clarification of its etiology, innovative studies are therefore indispensable. The aim of this paper is to review research on the pathogenesis of moyamoya disease to identify milestones in the direction of its true solution. Many hypotheses of the pathogenesis of moyamoya disease have been proposed in the past half century, including infection (viral and bacterial), autoimmune disorders, proteins abnormality, and gene abnormality. Some of these are now considered to be historical achievements. Others, however, can be still subjected to contemporary research. Currently, several genetic abnormalities are considered to offer the most probable hypothesis. In addition, interesting papers have been presented on the role of the endothelial progenitor cell on the pathogenesis of moyamoya disease. Intuitively, however, it appears that a single theory cannot always explain the pathogenesis of this disease adequately. In other words, the complex mechanism of several factors may comprehensively explain the formation of moyamoya disease. The "double hit hypothesis" is probably the best explanation for the complicated pathology and epidemiology of this disease.
Topics: Cerebral Arteries; Genetic Predisposition to Disease; Humans; Moyamoya Disease; Translational Research, Biomedical
PubMed: 22688062
DOI: 10.2176/nmc.52.267 -
Chinese Medical Journal Dec 2015To introduce the imaging characteristics of moyamoya disease (MMD) using high-resolution magnetic resonance imaging (HR-MRI) and to discuss the role of HR-MRI in... (Review)
Review
OBJECTIVE
To introduce the imaging characteristics of moyamoya disease (MMD) using high-resolution magnetic resonance imaging (HR-MRI) and to discuss the role of HR-MRI in differentiating MMD from other intracranial artery diseases, especially intracranial atherosclerotic disease (ICAD).
DATA SOURCES
This review was based on the data in articles published between 2005 and 2015, which were obtained from PubMed. The keywords included HR-MRI, MMD, ICAD, and intracranial artery diseases.
STUDY SELECTION
Articles related to HR-MRI for MMD or other intracranial artery diseases were selected for review.
RESULTS
There are differences between the characteristic patterns of HR-MRI in MMD and ICAD. MMD is associated with inward remodeling, smaller outer diameters, concentric occlusive lesions and homogeneous signal intensity, while ICAD is more likely to be associated with outward remodeling, normal outer diameters, eccentric occlusive lesions, and heterogeneous signal intensity. Other intracranial artery diseases, such as dissection and vasculitis, also have distinctive characteristics in HR-MRI. HR-MRI may become a useful tool for the differential diagnosis of MMD in the future.
CONCLUSIONS
HR-MRI of MMD provides a more in-depth understanding of MMD, and it is helpful in evaluating pathological changes in the vessel wall and in differentiating MMD from other intracranial artery steno-occlusive diseases, particularly ICAD.
Topics: Animals; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Middle Cerebral Artery; Moyamoya Disease
PubMed: 26612300
DOI: 10.4103/0366-6999.170257 -
Journal of Cerebral Blood Flow and... Apr 2017This study aimed to determine whether measurements of cerebrovascular reserve and oxygenation, assessed with spin relaxation rate R2', yield similar information about...
This study aimed to determine whether measurements of cerebrovascular reserve and oxygenation, assessed with spin relaxation rate R2', yield similar information about pathology in pre-operative Moyamoya disease patients, and to assess whether R2' is a better measure of oxygenation than other proposed markers, such as R2* and R2. Twenty-five pre-operative Moyamoya disease patients were scanned at 3.0T with acetazolamide challenge. Cerebral blood flow mapping with multi-delay arterial spin labeling, and R2*, R2, and R2' mapping with Gradient-Echo Sampling of Free Induction Decay and Echo were performed. No baseline cerebral blood flow difference was found between angiographically abnormal and normal regions (49 ± 12 vs. 48 ± 11 mL/100 g/min, p = 0.44). However, baseline R2' differed between these regions (3.2 ± 0.7 vs. 2.9 ± 0.6 s, p < 0.001), indicating reduced oxygenation in abnormal regions. Cerebrovascular reserve was lower in angiographically abnormal regions (21 ± 38 vs. 41 ± 26%, p = 0.001). All regions showed trend toward significantly improved oxygenation post-acetazolamide. Regions with poorer cerebrovascular reserve had lower baseline oxygenation (Kendall's τ = -0.24, p = 0.003). A number of angiographically abnormal regions demonstrated preserved cerebrovascular reserve, likely due to the presence of collaterals. Finally, of the concurrently measured relaxation rates, R2' was superior for oxygenation assessment.
Topics: Adult; Aged; Brain; Cerebrovascular Circulation; Female; Humans; Image Interpretation, Computer-Assisted; Magnetic Resonance Angiography; Male; Middle Aged; Moyamoya Disease; Oxygen; Prospective Studies; Young Adult
PubMed: 27207169
DOI: 10.1177/0271678X16651088 -
Revista de NeurologiaTo describe the clinical manifestations and to summarise the knowledge currently available about the epidemiology, physiopathology, histopathology, diagnosis and... (Review)
Review
AIMS
To describe the clinical manifestations and to summarise the knowledge currently available about the epidemiology, physiopathology, histopathology, diagnosis and treatment of moyamoya disease.
DEVELOPMENT
Moyamoya disease is produced by the spontaneous, progressive occlusion of the circle of Willis with the simultaneous appearance of natural intracranial and extra-intracranial collaterals. Both children and adults can be affected and it is to be found not only in Asia but also in Europe and America, although the frequency is unknown. Its primary lesion is a thickening of the tunica interna in the distal segment of the internal carotids, initially within the anterior portion of the circle of Willis. This results in the appearance of collateral vessels, which are first intracranial (moyamoya vessels) and then extracranial, to offset the critical reduction in regional cerebral blood flow. The most common clinical manifestation is motor-type ischaemic episodes, although it may also be accompanied by intracerebral haemorrhages. It can be diagnosed by means of suitable morphological and functional studies, such as cerebral pan angiography and xenon-CT, in patients with a founded clinical suspicion. Neurosurgical revascularisation is to date the most widely accepted treatment and can bring about a permanent clinical cure.
CONCLUSIONS
Moyamoya disease is a rare pathology that can be successfully treated using cerebral revascularisation, provided a timely diagnosis is reached and in indicated patients. Such therapy makes it possible to prevent the repetition of ischaemic events or haemorrhages that usually bring about severe limitations in the individual's personal development.
Topics: Cerebral Revascularization; Cerebrovascular Circulation; Diagnosis, Differential; Humans; Moyamoya Disease
PubMed: 16941427
DOI: No ID Found -
Orphanet Journal of Rare Diseases Jul 2023At present, the etiology of moyamoya disease is not clear, and it is necessary to explore the mechanism of its occurrence and development. Although some bulk sequencing...
BACKGROUND
At present, the etiology of moyamoya disease is not clear, and it is necessary to explore the mechanism of its occurrence and development. Although some bulk sequencing data have previously revealed transcriptomic changes in Moyamoya disease, single-cell sequencing data has been lacking.
METHODS
Two DSA(Digital Subtraction Angiography)-diagnosed patients with moyamoya disease were recruited between January 2021 and December 2021. Their peripheral blood samples were single-cell sequenced. CellRanger(10 x Genomics, version 3.0.1) was used to process the raw data, demultiplex cellular barcodes, map reads to the transcriptome, and dowm-sample reads(as required to generate normalized aggregate data across samples). There were 4 normal control samples, including two normal samples GSM5160432 and GSM5160434 of GSE168732, and two normal samples of GSE155698, namely GSM4710726 and GSM4710727. Weighted co-expression network analysis was used to explore the gene sets associated with moyamoya disease. GO analysis and KEGG analysis were used to explore gene enrichment pathways. Pseudo-time series analysis and cell interaction analysis were used to explore cell differentiation and cell interaction.
RESULTS
For the first time, we present a peripheral blood single cell sequencing landscape of Moyamoya disease, revealing cellular heterogeneity and gene expression heterogeneity. In addition, by combining with WGCNA analysis in public database and taking intersection, the key genes in moyamoya disease were obtained. namely PTP4A1, SPINT2, CSTB, PLA2G16, GPX1, HN1, LGALS3BP, IFI6, NDRG1, GOLGA2, LGALS3. Moreover, pseudo-time series analysis and cell interaction analysis revealed the differentiation of immune cells and the relationship between immune cells in Moyamoya disease.
CONCLUSIONS
Our study can provide information for the diagnosis and treatment of moyamoya disease.
Topics: Humans; Moyamoya Disease; Gene Expression Profiling; Angiography, Digital Subtraction; Transcriptome; Membrane Glycoproteins
PubMed: 37400835
DOI: 10.1186/s13023-023-02781-8 -
Scientific Reports Sep 2023Angiogenic factors associated with Moyamoya disease (MMD) are overexpressed in M2 polarized microglia in ischemic stroke, suggesting that microglia may be involved in...
Angiogenic factors associated with Moyamoya disease (MMD) are overexpressed in M2 polarized microglia in ischemic stroke, suggesting that microglia may be involved in the pathophysiology of MMD; however, existing approaches are not applicable to explore this hypothesis. Herein we applied blood induced microglial-like (iMG) cells. We recruited 25 adult patients with MMD and 24 healthy volunteers. Patients with MMD were subdivided into progressive (N = 7) or stable (N = 18) group whether novel symptoms or radiographic advancement of Suzuki stage within 1 year was observed or not. We produced 3 types of iMG cells; resting, M1-, and M2-induced cells from monocytes, then RNA sequencing followed by GO and KEGG pathway enrichment analysis and qPCR assay were performed. RNA sequencing of M2-induced iMG cells revealed that 600 genes were significantly upregulated (338) or downregulated (262) in patients with MMD. Inflammation and immune-related factors and angiogenesis-related factors were specifically associated with MMD in GO analysis. qPCR for MMP9, VEGFA, and TGFB1 expression validated these findings. This study is the first to demonstrate that M2 microglia may be involved in the angiogenic process of MMD. The iMG technique provides a promising approach to explore the bioactivity of microglia in cerebrovascular diseases.
Topics: Adult; Humans; Moyamoya Disease; Microglia; Inflammation; Cardiovascular Physiological Phenomena
PubMed: 37684266
DOI: 10.1038/s41598-023-41456-z -
International Journal of Medical... 2016Moyamoya disease (MMD) involves progressive occlusion of the intracranial internal carotid artery resulting in formation of moyamoya-like vessels at the base of the... (Review)
Review
Moyamoya disease (MMD) involves progressive occlusion of the intracranial internal carotid artery resulting in formation of moyamoya-like vessels at the base of the brain. It can be characterized by hemorrhage or ischemia. Direct vascular bypass is the main and most effective treatment of MMD. However, patients with MMD differ from those with normal cerebral vessels. MMD patients have unstable intracranial artery hemodynamics and a poor blood flow reserve; therefore, during the direct bypass of superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis, perioperative risk factors and anesthesia can affect the hemodynamics of these patients. When brain tissue cannot tolerate a high blood flow rate, it becomes prone to hyperperfusion syndrome, which leads to neurological function defects and can even cause intracranial hemorrhage in severe cases. The brain tissue is prone to infarction when hemodynamic equilibrium is affected. In addition, bypass vessels become susceptible to occlusion or atrophy when blood resistance increases. Even compression of the temporalis affects bypass vessels. Because the STA is used in MMD surgery, the scalp becomes ischemic and is likely to develop necrosis and infection. These complications of MMD surgery are difficult to manage and are not well understood. To date, no systematic studies of the complications that occur after direct bypass in MMD have been performed, and reported complications are hidden among various case studies; therefore, this paper presents a review and summary of the literature in PubMed on the complications of direct bypass in MMD.
Topics: Brain; Carotid Artery, Internal; Cerebral Revascularization; Humans; Intracranial Hemorrhages; Middle Cerebral Artery; Moyamoya Disease; Temporal Arteries; Treatment Outcome
PubMed: 27499690
DOI: 10.7150/ijms.15390