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Kiyohiro Houkin - One of the best experts on this subject based on the ideXlab platform.

  • Predictive factors for acute thrombogenesis occurring immediately after bypass procedure for Moyamoya Disease
    Neurosurgical Review, 2019
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Hime Suzuki, Katsuya Komatsu, Yukinori Akiyama, Masahiko Wanibuchi, Ryo Ukai, Nobuhiro Mikuni
    Abstract:

    Extracranial-to-intracranial (EC-IC) bypass surgery is an effective treatment for patients with Moyamoya Disease and other conditions. Some patients with Moyamoya Disease have a risk of acute thrombogenesis at the anastomotic site just after bypass surgery. The purpose of this study was to study risk factors of acute thrombogenesis and determine effective countermeasures. This study included 48 patients (66 EC-IC bypass procedures) with Moyamoya Disease and 52 controls (54 procedures) without Moyamoya Disease. The development of acute thrombogenesis was compared between the Moyamoya Disease and control groups. In the Moyamoya Disease group, clinical and radiological characteristics were assessed with respect to acute thrombogenesis. In the patients with acute thrombogenesis, causes of technical problems were retrospectively examined. The incidence of acute thrombogenesis was significantly higher in the Moyamoya Disease group than those in the control group. In the Moyamoya Disease group, acute thrombogenesis was observed in seven patients. In the Moyamoya Disease group, the magnetic resonance angiography (MRA) scores were significantly higher in patients with acute thrombogenesis than those in the patients without acute thrombogenesis. In the multivariate analysis, the predictive factor of acute thrombogenesis in Moyamoya Disease was a high MRA score (odds ratio, 2.336; p  = 0.009). During EC-IC bypass surgery for Moyamoya Disease, acute thrombogenesis should be considered to obtain a high patency rate, particularly in patients with high MRA scores. Acute thrombogenesis will not influence morbidity if proper countermeasures are followed; therefore, the prediction and recognition of white thrombus are important for a successful bypass surgery.

  • meandering flow void around the splenium in Moyamoya Disease
    Neurological Research, 2017
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Katsuya Komatsu, Masahiko Wanibuchi, Tomoyoshi Kuribara, Yusuke Kimura, Nobuhiro Mikuni
    Abstract:

    AbstractObjectives: Meandering flow void around the splenium, which can be recognized on conventional T2-weighted images, implicates collateral flow in the splenial artery in patients with Moyamoya Disease. In this report, curves of flow voids around the splenium (SFVs) were evaluated in patients with Moyamoya Disease, and their diagnostic value and pathophysiology were verified.Methods: A total of 65 consecutive patients with Moyamoya Disease were included in this analysis. The number of SFV curves was counted on each side. The numbers of SFV curves in patients with Moyamoya Disease was compared with those in the control group. The clinical features of patients with large numbers of flow voids were analyzed.Results: The mean number of SFVs was significantly higher in the Moyamoya Disease group than in the control group or the atherosclerotic Disease group. The cut-off SFV count for a diagnosis of Moyamoya Disease should be set to 2.25 in consideration of receiver operating characteristic curve analysis. ...

  • Predictive factors for epilepsy in Moyamoya Disease
    Journal of Stroke and Cerebrovascular Diseases, 2015
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Yukinori Akiyama, Masahiko Wanibuchi, Satoko Ochi, Nobuhiro Mikuni
    Abstract:

    Background Epilepsy cannot always be recognized in patients with Moyamoya Disease. In this report, the clinical features of patients with epilepsy were evaluated for assessing the predictive factors of epilepsy in Moyamoya Disease. Methods A total of 64 consecutive patients with Moyamoya Disease were included in this study. During their follow-up periods, 7 patients were diagnosed with epilepsy. Then, the patients with epilepsy were compared with the patients without epilepsy regarding their clinical features. Results Analysis of patient background characteristics revealed a significantly higher incidence of epilepsy in patients with high modified Rankin Scale (mRS) scores, high cerebrovascular attack scores, onset age of 3 years or less, early seizures, cortical involvement, stroke subtype, and diffuse brain atrophy. A logistic analysis of epilepsy data revealed significant differences between the 2 groups in mRS score, cerebrovascular attack score, onset age 3 years or less, early seizure, cortical involvement, stroke subtype, and diffuse brain atrophy. Of these, significant differences were noted in 3 items (mRS score, early seizure, and diffuse brain atrophy) on multivariate analysis. These 3 items were selected as the basis of our new Moyamoya Disease epilepsy risk scale (MDERS), which we then evaluated. The cutoff value estimated by the receiver operating characteristic curve was set at 1 (sensitivity, .857; specificity, .825) or 2 (sensitivity, .571; specificity, 1.000). Conclusions Epilepsy in Moyamoya Disease is associated with clinical factors and is not an independent category. For prediction of epilepsy in Moyamoya Disease, MDERS is a simple and convenient assessment scale.

  • Moyamoya Disease current concepts and future perspectives
    Lancet Neurology, 2008
    Co-Authors: Satoshi Kuroda, Kiyohiro Houkin
    Abstract:

    Summary Moyamoya Disease is an uncommon cerebrovascular Disease that is characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. The Disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed Moyamoya vessels. The incidence of Moyamoya Disease is high in east Asia, and familial forms account for about 15% of patients with this Disease. Moyamoya Disease has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years. Most paediatric patients have ischaemic attacks, whereas adult patients can have ischaemic attacks, intracranial bleeding, or both. Extracranial–intracranial arterial bypass, including anastomosis of the superficial temporal artery to the middle cerebral artery and indirect bypass, can help prevent further ischaemic attacks, although the beneficial effect on haemorrhagic stroke is still not clear. In this Review, we summarise the epidemiology, aetiology, clinical features, diagnosis, surgical treatment, and outcomes of Moyamoya Disease. Recent updates and future perspectives for Moyamoya Disease will also be discussed.

  • novel epidemiological features of Moyamoya Disease
    Journal of Neurology Neurosurgery and Psychiatry, 2008
    Co-Authors: Takeo Baba, Kiyohiro Houkin, Satoshi Kuroda
    Abstract:

    Background: Many clinical features that are specific to Moyamoya Disease have been reported and cited in textbooks based on previous data. The purpose of this study is to investigate the present epidemiological features of Moyamoya Disease based on recently obtained regional all-inclusive data. Methods: The authors performed an all-inclusive survey of Moyamoya Disease in Hokkaido, one of the major islands in Japan that has a population of 5.63 million. The epidemiological features were analysed based on the data from 267 newly registered patients with Moyamoya Disease in Hokkaido from 2002 to 2006. These analysed data were adjusted to the whole Japanese population at 2005. Results: The detection rate of the Disease per year was 0.94 patients per 100 000 people, and prevalence was 10.5 patients per 100 000 people. The incidence of ischaemia concerned with the Disease was 0.53 patients per 100 000 people-years and haemorrhage was 0.2 patients per 100 000 people-years. The ratio of female to male patients was 2.18. The ratio of patients aged 10 years and above to under 10 years of age at onset was 6.18. Two peaks for age of onset were seen: the highest was observed between 45 and 49 years, and the second between 5 and 9 years. Asymptomatic patients comprised 17.8% of the total number of patients. Conclusion: The epidemiological features of Moyamoya Disease determined by this survey varied considerably from previous data. The detection rate and prevalence of the Disease were higher than those reported previously. The highest peak of onset age was older than those reported previously. In addition, it was revealed that asymptomatic Moyamoya patients are not always rare in Japan.

Nobuhiro Mikuni - One of the best experts on this subject based on the ideXlab platform.

  • Predictive factors for acute thrombogenesis occurring immediately after bypass procedure for Moyamoya Disease
    Neurosurgical Review, 2019
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Hime Suzuki, Katsuya Komatsu, Yukinori Akiyama, Masahiko Wanibuchi, Ryo Ukai, Nobuhiro Mikuni
    Abstract:

    Extracranial-to-intracranial (EC-IC) bypass surgery is an effective treatment for patients with Moyamoya Disease and other conditions. Some patients with Moyamoya Disease have a risk of acute thrombogenesis at the anastomotic site just after bypass surgery. The purpose of this study was to study risk factors of acute thrombogenesis and determine effective countermeasures. This study included 48 patients (66 EC-IC bypass procedures) with Moyamoya Disease and 52 controls (54 procedures) without Moyamoya Disease. The development of acute thrombogenesis was compared between the Moyamoya Disease and control groups. In the Moyamoya Disease group, clinical and radiological characteristics were assessed with respect to acute thrombogenesis. In the patients with acute thrombogenesis, causes of technical problems were retrospectively examined. The incidence of acute thrombogenesis was significantly higher in the Moyamoya Disease group than those in the control group. In the Moyamoya Disease group, acute thrombogenesis was observed in seven patients. In the Moyamoya Disease group, the magnetic resonance angiography (MRA) scores were significantly higher in patients with acute thrombogenesis than those in the patients without acute thrombogenesis. In the multivariate analysis, the predictive factor of acute thrombogenesis in Moyamoya Disease was a high MRA score (odds ratio, 2.336; p  = 0.009). During EC-IC bypass surgery for Moyamoya Disease, acute thrombogenesis should be considered to obtain a high patency rate, particularly in patients with high MRA scores. Acute thrombogenesis will not influence morbidity if proper countermeasures are followed; therefore, the prediction and recognition of white thrombus are important for a successful bypass surgery.

  • Influence of Inflammatory Disease on the Pathophysiology of Moyamoya Disease and Quasi-Moyamoya Disease
    Neurologia medico-chirurgica, 2019
    Co-Authors: Takeshi Mikami, Hime Suzuki, Katsuya Komatsu, Nobuhiro Mikuni
    Abstract:

    Moyamoya Disease is a unique cerebrovascular Disease that is characterized by progressive bilateral stenotic alteration at the terminal portion of the internal carotid arteries. These changes induce the formation of an abnormal vascular network composed of collateral pathways known as Moyamoya vessels. In quasi-Moyamoya Disease, a similar stenotic vascular abnormality is associated with an underlying Disease, which is sometimes an inflammatory Disease. Recent advances in Moyamoya Disease research implicate genetic background and immunological mediators, and postulate an association with inflammatory Disease as a cause of, or progressive factor in, quasi-Moyamoya Disease. Although this Disease has well-defined clinical and radiological characteristics, the role of inflammation has not been rigorously explored. Herein, we focused on reviewing two main themes: (1) molecular biology of inflammation in Moyamoya Disease, and (2) clinical significance of inflammation in quasi-Moyamoya Disease. We have summarized the findings of the former theme according to the following topics: (1) inflammatory biomarkers, (2) genetic background of inflammatory response, (3) endothelial progenitor cells, and (4) noncoding ribonucleic acids. Under the latter theme, we summarized the findings according to the following topics: (1) influence of inflammatory Disease, (2) vascular remodeling, and (3) mechanisms gleaned from clinical cases. This review includes articles published up to February 2019 and provides novel insights for the treatment of the Moyamoya Disease and quasi-Moyamoya Disease.

  • meandering flow void around the splenium in Moyamoya Disease
    Neurological Research, 2017
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Katsuya Komatsu, Masahiko Wanibuchi, Tomoyoshi Kuribara, Yusuke Kimura, Nobuhiro Mikuni
    Abstract:

    AbstractObjectives: Meandering flow void around the splenium, which can be recognized on conventional T2-weighted images, implicates collateral flow in the splenial artery in patients with Moyamoya Disease. In this report, curves of flow voids around the splenium (SFVs) were evaluated in patients with Moyamoya Disease, and their diagnostic value and pathophysiology were verified.Methods: A total of 65 consecutive patients with Moyamoya Disease were included in this analysis. The number of SFV curves was counted on each side. The numbers of SFV curves in patients with Moyamoya Disease was compared with those in the control group. The clinical features of patients with large numbers of flow voids were analyzed.Results: The mean number of SFVs was significantly higher in the Moyamoya Disease group than in the control group or the atherosclerotic Disease group. The cut-off SFV count for a diagnosis of Moyamoya Disease should be set to 2.25 in consideration of receiver operating characteristic curve analysis. ...

  • Geometrical Complexity of Cortical Microvascularization in Moyamoya Disease.
    World Neurosurgery, 2017
    Co-Authors: Katsuya Komatsu, Takeshi Mikami, Hime Suzuki, Yukinori Akiyama, Rei Enatsu, Masahiko Wanibuchi, Nobuhiro Mikuni
    Abstract:

    Background Dilatation of the microvascular diameter is recognized in Moyamoya Disease and referred to as microvascularization. The purpose of this study was to characterize the cortical microvascularization in Moyamoya Disease using imaging analysis, and to explore the developmental mechanism of the collateral network around the cortical surface. Methods A total of 20 hemispheric sides of 14 patients with Moyamoya Disease were included in this study. From the intraoperative images, cortical surface images were extracted, and binary images were subsequently created. Then the ratio of the microvessels of the brain surface (vascular fraction; VF) and the box-counting fractal dimension (Db) values were calculated. The VF and Db values in the Moyamoya Disease group were then compared with those in atherosclerotic Disease and nonischemic Disease groups, and assessed in terms of clinical and radiologic factors. Results VF was significantly higher in the Moyamoya Disease group compared with the atherosclerotic Disease group, and Db was significantly higher in the Moyamoya Disease group compared with the atherosclerotic Disease and nonischemic Disease groups. In the Moyamoya Disease group, VF showed a moderate correlation with magnetic resonance angiography (MRA) score. Moreover, Db was significantly higher in the pediatric patients, in the presence of ischemic symptoms, and in the presence of ivy sign, and Db showed a moderate correlation with MRA score and cerebral blood flow in Moyamoya Disease. Conclusions In the patients with Moyamoya Disease, the cortical microvascularization exhibited increased Db and dilatation of the pial arteries. In Moyamoya Disease, cortical microvascularization is associated with clinical and radiologic factors. This microvascularization might be a compensatory mechanism in the ischemic condition in Moyamoya Disease.

  • Predictive factors for epilepsy in Moyamoya Disease
    Journal of Stroke and Cerebrovascular Diseases, 2015
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Yukinori Akiyama, Masahiko Wanibuchi, Satoko Ochi, Nobuhiro Mikuni
    Abstract:

    Background Epilepsy cannot always be recognized in patients with Moyamoya Disease. In this report, the clinical features of patients with epilepsy were evaluated for assessing the predictive factors of epilepsy in Moyamoya Disease. Methods A total of 64 consecutive patients with Moyamoya Disease were included in this study. During their follow-up periods, 7 patients were diagnosed with epilepsy. Then, the patients with epilepsy were compared with the patients without epilepsy regarding their clinical features. Results Analysis of patient background characteristics revealed a significantly higher incidence of epilepsy in patients with high modified Rankin Scale (mRS) scores, high cerebrovascular attack scores, onset age of 3 years or less, early seizures, cortical involvement, stroke subtype, and diffuse brain atrophy. A logistic analysis of epilepsy data revealed significant differences between the 2 groups in mRS score, cerebrovascular attack score, onset age 3 years or less, early seizure, cortical involvement, stroke subtype, and diffuse brain atrophy. Of these, significant differences were noted in 3 items (mRS score, early seizure, and diffuse brain atrophy) on multivariate analysis. These 3 items were selected as the basis of our new Moyamoya Disease epilepsy risk scale (MDERS), which we then evaluated. The cutoff value estimated by the receiver operating characteristic curve was set at 1 (sensitivity, .857; specificity, .825) or 2 (sensitivity, .571; specificity, 1.000). Conclusions Epilepsy in Moyamoya Disease is associated with clinical factors and is not an independent category. For prediction of epilepsy in Moyamoya Disease, MDERS is a simple and convenient assessment scale.

Takeshi Mikami - One of the best experts on this subject based on the ideXlab platform.

  • Predictive factors for acute thrombogenesis occurring immediately after bypass procedure for Moyamoya Disease
    Neurosurgical Review, 2019
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Hime Suzuki, Katsuya Komatsu, Yukinori Akiyama, Masahiko Wanibuchi, Ryo Ukai, Nobuhiro Mikuni
    Abstract:

    Extracranial-to-intracranial (EC-IC) bypass surgery is an effective treatment for patients with Moyamoya Disease and other conditions. Some patients with Moyamoya Disease have a risk of acute thrombogenesis at the anastomotic site just after bypass surgery. The purpose of this study was to study risk factors of acute thrombogenesis and determine effective countermeasures. This study included 48 patients (66 EC-IC bypass procedures) with Moyamoya Disease and 52 controls (54 procedures) without Moyamoya Disease. The development of acute thrombogenesis was compared between the Moyamoya Disease and control groups. In the Moyamoya Disease group, clinical and radiological characteristics were assessed with respect to acute thrombogenesis. In the patients with acute thrombogenesis, causes of technical problems were retrospectively examined. The incidence of acute thrombogenesis was significantly higher in the Moyamoya Disease group than those in the control group. In the Moyamoya Disease group, acute thrombogenesis was observed in seven patients. In the Moyamoya Disease group, the magnetic resonance angiography (MRA) scores were significantly higher in patients with acute thrombogenesis than those in the patients without acute thrombogenesis. In the multivariate analysis, the predictive factor of acute thrombogenesis in Moyamoya Disease was a high MRA score (odds ratio, 2.336; p  = 0.009). During EC-IC bypass surgery for Moyamoya Disease, acute thrombogenesis should be considered to obtain a high patency rate, particularly in patients with high MRA scores. Acute thrombogenesis will not influence morbidity if proper countermeasures are followed; therefore, the prediction and recognition of white thrombus are important for a successful bypass surgery.

  • Influence of Inflammatory Disease on the Pathophysiology of Moyamoya Disease and Quasi-Moyamoya Disease
    Neurologia medico-chirurgica, 2019
    Co-Authors: Takeshi Mikami, Hime Suzuki, Katsuya Komatsu, Nobuhiro Mikuni
    Abstract:

    Moyamoya Disease is a unique cerebrovascular Disease that is characterized by progressive bilateral stenotic alteration at the terminal portion of the internal carotid arteries. These changes induce the formation of an abnormal vascular network composed of collateral pathways known as Moyamoya vessels. In quasi-Moyamoya Disease, a similar stenotic vascular abnormality is associated with an underlying Disease, which is sometimes an inflammatory Disease. Recent advances in Moyamoya Disease research implicate genetic background and immunological mediators, and postulate an association with inflammatory Disease as a cause of, or progressive factor in, quasi-Moyamoya Disease. Although this Disease has well-defined clinical and radiological characteristics, the role of inflammation has not been rigorously explored. Herein, we focused on reviewing two main themes: (1) molecular biology of inflammation in Moyamoya Disease, and (2) clinical significance of inflammation in quasi-Moyamoya Disease. We have summarized the findings of the former theme according to the following topics: (1) inflammatory biomarkers, (2) genetic background of inflammatory response, (3) endothelial progenitor cells, and (4) noncoding ribonucleic acids. Under the latter theme, we summarized the findings according to the following topics: (1) influence of inflammatory Disease, (2) vascular remodeling, and (3) mechanisms gleaned from clinical cases. This review includes articles published up to February 2019 and provides novel insights for the treatment of the Moyamoya Disease and quasi-Moyamoya Disease.

  • meandering flow void around the splenium in Moyamoya Disease
    Neurological Research, 2017
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Katsuya Komatsu, Masahiko Wanibuchi, Tomoyoshi Kuribara, Yusuke Kimura, Nobuhiro Mikuni
    Abstract:

    AbstractObjectives: Meandering flow void around the splenium, which can be recognized on conventional T2-weighted images, implicates collateral flow in the splenial artery in patients with Moyamoya Disease. In this report, curves of flow voids around the splenium (SFVs) were evaluated in patients with Moyamoya Disease, and their diagnostic value and pathophysiology were verified.Methods: A total of 65 consecutive patients with Moyamoya Disease were included in this analysis. The number of SFV curves was counted on each side. The numbers of SFV curves in patients with Moyamoya Disease was compared with those in the control group. The clinical features of patients with large numbers of flow voids were analyzed.Results: The mean number of SFVs was significantly higher in the Moyamoya Disease group than in the control group or the atherosclerotic Disease group. The cut-off SFV count for a diagnosis of Moyamoya Disease should be set to 2.25 in consideration of receiver operating characteristic curve analysis. ...

  • Geometrical Complexity of Cortical Microvascularization in Moyamoya Disease.
    World Neurosurgery, 2017
    Co-Authors: Katsuya Komatsu, Takeshi Mikami, Hime Suzuki, Yukinori Akiyama, Rei Enatsu, Masahiko Wanibuchi, Nobuhiro Mikuni
    Abstract:

    Background Dilatation of the microvascular diameter is recognized in Moyamoya Disease and referred to as microvascularization. The purpose of this study was to characterize the cortical microvascularization in Moyamoya Disease using imaging analysis, and to explore the developmental mechanism of the collateral network around the cortical surface. Methods A total of 20 hemispheric sides of 14 patients with Moyamoya Disease were included in this study. From the intraoperative images, cortical surface images were extracted, and binary images were subsequently created. Then the ratio of the microvessels of the brain surface (vascular fraction; VF) and the box-counting fractal dimension (Db) values were calculated. The VF and Db values in the Moyamoya Disease group were then compared with those in atherosclerotic Disease and nonischemic Disease groups, and assessed in terms of clinical and radiologic factors. Results VF was significantly higher in the Moyamoya Disease group compared with the atherosclerotic Disease group, and Db was significantly higher in the Moyamoya Disease group compared with the atherosclerotic Disease and nonischemic Disease groups. In the Moyamoya Disease group, VF showed a moderate correlation with magnetic resonance angiography (MRA) score. Moreover, Db was significantly higher in the pediatric patients, in the presence of ischemic symptoms, and in the presence of ivy sign, and Db showed a moderate correlation with MRA score and cerebral blood flow in Moyamoya Disease. Conclusions In the patients with Moyamoya Disease, the cortical microvascularization exhibited increased Db and dilatation of the pial arteries. In Moyamoya Disease, cortical microvascularization is associated with clinical and radiologic factors. This microvascularization might be a compensatory mechanism in the ischemic condition in Moyamoya Disease.

  • Predictive factors for epilepsy in Moyamoya Disease
    Journal of Stroke and Cerebrovascular Diseases, 2015
    Co-Authors: Takeshi Mikami, Kiyohiro Houkin, Yukinori Akiyama, Masahiko Wanibuchi, Satoko Ochi, Nobuhiro Mikuni
    Abstract:

    Background Epilepsy cannot always be recognized in patients with Moyamoya Disease. In this report, the clinical features of patients with epilepsy were evaluated for assessing the predictive factors of epilepsy in Moyamoya Disease. Methods A total of 64 consecutive patients with Moyamoya Disease were included in this study. During their follow-up periods, 7 patients were diagnosed with epilepsy. Then, the patients with epilepsy were compared with the patients without epilepsy regarding their clinical features. Results Analysis of patient background characteristics revealed a significantly higher incidence of epilepsy in patients with high modified Rankin Scale (mRS) scores, high cerebrovascular attack scores, onset age of 3 years or less, early seizures, cortical involvement, stroke subtype, and diffuse brain atrophy. A logistic analysis of epilepsy data revealed significant differences between the 2 groups in mRS score, cerebrovascular attack score, onset age 3 years or less, early seizure, cortical involvement, stroke subtype, and diffuse brain atrophy. Of these, significant differences were noted in 3 items (mRS score, early seizure, and diffuse brain atrophy) on multivariate analysis. These 3 items were selected as the basis of our new Moyamoya Disease epilepsy risk scale (MDERS), which we then evaluated. The cutoff value estimated by the receiver operating characteristic curve was set at 1 (sensitivity, .857; specificity, .825) or 2 (sensitivity, .571; specificity, 1.000). Conclusions Epilepsy in Moyamoya Disease is associated with clinical factors and is not an independent category. For prediction of epilepsy in Moyamoya Disease, MDERS is a simple and convenient assessment scale.

Adnan I. Qureshi - One of the best experts on this subject based on the ideXlab platform.

  • prevalence and characteristics of concurrent down syndrome in patients with Moyamoya Disease
    Neurosurgery, 2013
    Co-Authors: Daraspreet Kainth, Saqib A. Chaudhry, Hunar Kainth, Fareed K Suri, Adnan I. Qureshi
    Abstract:

    BACKGROUND: An association between Moyamoya Disease and Down syndrome appears to exist on the basis of reported anecdotal cases in the literature. OBJECTIVE: To determine the prevalence of Down syndrome associated with Moyamoya Disease in inpatients and to identify the demographic and clinical features of Moyamoya Disease that may be unique when associated with Down syndrome. METHODS: In this observational study, we analyzed data from the Nationwide Inpatient Sample between 2002 and 2009 using International Classification of Diseases codes for Moyamoya Disease and Down syndrome for patient identification. Data including patient age, sex, race/ethnicity, secondary diagnosis, procedures, hospital costs, and patient outcomes were obtained. RESULTS: From 2002 to 2009, an estimated 518 patients (mean ± SD age, 16.2 ± 1.68 years) with coexisting Moyamoya Disease and Down syndrome were admitted. The estimated prevalence was 3.8% (3760 per 100,000) among patients admitted with Moyamoya Disease and 9.5% (9540 per 100,000) among Moyamoya patients < 15 years of age. Patients admitted with Moyamoya Disease and Down syndrome were most frequently white and Hispanic (P = .02). They were more likely to present with ischemic stroke and less commonly with hemorrhagic stroke (15.3% and 2.7%, respectively; P < .05). CONCLUSION: This is the first study to estimate the prevalence of Down syndrome in patients with Moyamoya Disease. The 26-fold-greater prevalence of Down syndrome in patients with coexisting Moyamoya Disease compared with the prevalence of Down syndrome among live births (145 per 100,000) highlights the need for a better understanding of the common pathophysiology of the 2 conditions.

  • Epidemiological and clinical features of Moyamoya Disease in the USA.
    Neuroepidemiology, 2013
    Co-Authors: Daraspreet Kainth, Saqib A. Chaudhry, Hunar Kainth, Fareed K Suri, Adnan I. Qureshi
    Abstract:

    Background: An increasing number of cases of Moyamoya Disease have been reported in the Japanese and US literature. We performed this study to quantify the rise in the prevalence of Moyamoya Disease and to study the unique epidemiological and clinical features in the USA that may explain a change in incidence. Methods: We analyzed data derived from patients entered in the Nationwide Inpatient Sample between 2005 and 2008, using ICD-9 codes for Moyamoya Disease. Data including patient age, gender, ethnicity, secondary diagnosis, medical complications, and hospital costs were obtained. Results: From 2005 to 2008 in the USA, there were an estimated 7,473 patients admitted with a primary or secondary diagnosis of Moyamoya Disease. Patients admitted with Moyamoya Disease were most frequently women and Caucasian. Overall, ischemic stroke was the most common reason for admission. Hemorrhagic stroke was more frequent in adults compared with children, 18.1 versus 1.5% (p Conclusion: The number of patients identified and admitted with Moyamoya Disease has risen dramatically in the last decade. This study can lead to a better understanding of the Disease pattern and healthcare consequences in the USA and suggests that pathophysiologic differences in Moyamoya Disease may exist.

Satoshi Kuroda - One of the best experts on this subject based on the ideXlab platform.

  • Moyamoya Disease current concepts and future perspectives
    Lancet Neurology, 2008
    Co-Authors: Satoshi Kuroda, Kiyohiro Houkin
    Abstract:

    Summary Moyamoya Disease is an uncommon cerebrovascular Disease that is characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. The Disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed Moyamoya vessels. The incidence of Moyamoya Disease is high in east Asia, and familial forms account for about 15% of patients with this Disease. Moyamoya Disease has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years. Most paediatric patients have ischaemic attacks, whereas adult patients can have ischaemic attacks, intracranial bleeding, or both. Extracranial–intracranial arterial bypass, including anastomosis of the superficial temporal artery to the middle cerebral artery and indirect bypass, can help prevent further ischaemic attacks, although the beneficial effect on haemorrhagic stroke is still not clear. In this Review, we summarise the epidemiology, aetiology, clinical features, diagnosis, surgical treatment, and outcomes of Moyamoya Disease. Recent updates and future perspectives for Moyamoya Disease will also be discussed.

  • novel epidemiological features of Moyamoya Disease
    Journal of Neurology Neurosurgery and Psychiatry, 2008
    Co-Authors: Takeo Baba, Kiyohiro Houkin, Satoshi Kuroda
    Abstract:

    Background: Many clinical features that are specific to Moyamoya Disease have been reported and cited in textbooks based on previous data. The purpose of this study is to investigate the present epidemiological features of Moyamoya Disease based on recently obtained regional all-inclusive data. Methods: The authors performed an all-inclusive survey of Moyamoya Disease in Hokkaido, one of the major islands in Japan that has a population of 5.63 million. The epidemiological features were analysed based on the data from 267 newly registered patients with Moyamoya Disease in Hokkaido from 2002 to 2006. These analysed data were adjusted to the whole Japanese population at 2005. Results: The detection rate of the Disease per year was 0.94 patients per 100 000 people, and prevalence was 10.5 patients per 100 000 people. The incidence of ischaemia concerned with the Disease was 0.53 patients per 100 000 people-years and haemorrhage was 0.2 patients per 100 000 people-years. The ratio of female to male patients was 2.18. The ratio of patients aged 10 years and above to under 10 years of age at onset was 6.18. Two peaks for age of onset were seen: the highest was observed between 45 and 49 years, and the second between 5 and 9 years. Asymptomatic patients comprised 17.8% of the total number of patients. Conclusion: The epidemiological features of Moyamoya Disease determined by this survey varied considerably from previous data. The detection rate and prevalence of the Disease were higher than those reported previously. The highest peak of onset age was older than those reported previously. In addition, it was revealed that asymptomatic Moyamoya patients are not always rare in Japan.

  • Clinical features of familial Moyamoya Disease
    Child's Nervous System, 2005
    Co-Authors: Rina Nanba, Satoshi Kuroda, Kiyohiro Houkin, M Tada, Tatsuya Ishikawa, Yoshinobu Iwasaki
    Abstract:

    Objects This study aims to clarify the genetic background of Moyamoya Disease by comparing clinical features between familial and sporadic cases to reveal the responsible genes for familial Moyamoya Disease.

  • Cerebral revascularization for Moyamoya Disease
    Operative Techniques in Neurosurgery, 1999
    Co-Authors: Kiyohiro Houkin, Satoshi Kuroda
    Abstract:

    Eighty-four patients with Moyamoya Disease, including 36 pediatric cases and 48 adult cases, were treated by revascularization surgery. Ischemic onset is a good indication for surgical revascularization in both pediatric and adult cases. Combined surgery of superficial temporal artery-middle cerebral artery anastomosis and indirect revascularization of encephalo-duro-arterio-myo-synangiosis (EDAMS) or encephalo-duro-myo-arterio-pericranio-synangiosis (EDAMPS), which is a minor modification of EDAMS, was performed. Ischemic attacks disappeared in most patients within 1 year (mean) after surgery. However, in adult cases with hemorrhagic onset, rebleeding was seen in 6 of 48 cases (12.5%; mean follow-up, 6 years). Recent advances in clinical and basic studies of Moyamoya Disease are reviewed.

  • Angiographic Analysis of Moyamoya Disease : How Does Moyamoya Disease Progress?
    Neurologia medico-chirurgica, 1996
    Co-Authors: Kiyohiro Houkin, Satoshi Kuroda, Tetsuyuki Yoshimoto, Akihiro Takahashi, Tatsuya Ishikawa, Hiroshi Abe
    Abstract:

    Differences in the clinical presentation and angiographic stages of Moyamoya Disease were studied in 69 patients, 35 children (6.3 ± 2.9 years old) and 34 adults (44.6 ± 10.5 years old). The angiographic stage (Suzuki''s stage) was compared between childhood and adulthood, ischemic onset and hemorrhagic onset, and female and male. The distribution of the angiographic stage was not very different, but was significantly shifted from stage III to stage IV in adult cases. The angiographic stage in patients with hemorrhagic onset was not significantly different from those with ischemic onset. There was no significant difference between females and males. Angiographic change is not very remarkable between pediatric and adult Moyamoya Disease. The angiographic stage does not directly correlate with the distinct clinical presentations between pediatric and adult Moyamoya Disease. Other factors such as cerebral blood flow demand and arteriosclerotic change seem to cause these differences.