The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review
CONCLUSIONS: Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.PMID:34469862 | DOI:10.3171/2021.6.FOCUS21281 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Bhanu Jayanand Sudhir Arun Gowda Keelara Easwer Harihara Venkat Ken Kazumata Ananthalakshmy Sundararaman Source Type: research

Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E10. doi: 10.3171/2021.6.FOCUS21374.NO ABSTRACTPMID:34469863 | DOI:10.3171/2021.6.FOCUS21374 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Source Type: research

Introduction. Translational research advances in the evaluation and management of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E1. doi: 10.3171/2021.6.FOCUS21373.NO ABSTRACTPMID:34469864 | DOI:10.3171/2021.6.FOCUS21373 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Giuseppe Lanzino Gary K Steinberg Bin Xu Source Type: research

Experimental animal models for moyamoya disease and treatment: a pathogenesis-oriented scoping review
CONCLUSIONS: Models generated for MMD have involved three general approaches: surgical, immunological, and genetic. Although each reflects a key aspect of MMD pathogenesis, the failure of any individual model to recapitulate the development, progression, and consequences of the disease underscores the importance of future work in developing a multietiology model.PMID:34469865 | DOI:10.3171/2021.6.FOCUS21284 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Michael S Rallo Omar Akel Akhilesh Gurram Hai Sun Source Type: research

Experimental animal models for the study of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E4. doi: 10.3171/2021.5.FOCUS21282.ABSTRACTMoyamoya disease is a rare disorder of the cerebrovascular system affecting individuals in a bimodal age distribution and is characterized by progressive vascular stenosis of the bilateral supraclinoid internal carotid arteries with compensatory formation of collateral vessels at the base of the brain. Despite the disease's initial description in the literature in 1957, little progress has been made in the development of medical and surgical therapeutics due to, in no small part, the lack of effective experimental animal models. Currently, there is ...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Vijay Letchuman Leonel Ampie Panagiotis Mastorakos Daniel M S Raper Ryan T Kellogg Min S Park Source Type: research

The role of atorvastatin in collateral circulation formation induced by encephaloduroarteriosynangiosis: a prospective trial
CONCLUSIONS: The results of this prospective clinical trial have indicated that atorvastatin administered at 20 mg daily is safe and effective for the formation of postoperative collateral induced by EDAS.PMID:34469867 | DOI:10.3171/2021.6.FOCUS21112 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Qian-Nan Wang Xiang-Yang Bao Zheng-Xing Zou Xiao-Peng Wang Qian Zhang De-Sheng Li Ya-Qun Zhao Lian Duan Source Type: research

Motor excitability in bilateral moyamoya vasculopathy and the impact of revascularization
CONCLUSIONS: The study results suggested that, in the case of a bilateral chronic ischemia, a compensation mechanism between both hemispheres seemed to exist that normalized after revascularization surgery. A potential role of nTMS in predicting the efficacy of revascularization must be further assessed.PMID:34469868 | DOI:10.3171/2021.6.FOCUS21280 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Gueliz Acker Davide Giampiccolo Kerstin Rubarth Robert Mertens Anna Zdunczyk Juliane Hardt Daniel Jussen Heike Schneider Tizian Rosenstock Vera Mueller Thomas Picht Peter Vajkoczy Source Type: research

Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya
Neurosurg Focus. 2021 Sep;51(3):E8. doi: 10.3171/2021.6.FOCUS21283.ABSTRACTOBJECTIVE: Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya.METHODS: A retrospective surgical...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Joseph H Garcia Ramin A Morshed Ethan A Winkler Yi Li Christine K Fox Heather J Fullerton Caleb Rutledge Angad S Beniwal Michael T Lawton Adib A Abla Nalin Gupta Steven W Hetts Source Type: research

Transcriptome-wide analysis of intracranial artery in patients with moyamoya disease showing upregulation of immune response, and downregulation of oxidative phosphorylation and DNA repair
CONCLUSIONS: Transcriptome-wide analysis showed upregulation of genes for immune responses and downregulation of genes for DNA repair and oxidative phosphorylation within the intracranial artery of patients with MMD. These findings may represent clues to the pathophysiology of MMD.PMID:34469870 | DOI:10.3171/2021.6.FOCUS20870 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Fumiaki Kanamori Kinya Yokoyama Akinobu Ota Kazuhiro Yoshikawa Sivasundaram Karnan Mikio Maruwaka Kenzo Shimizu Shinji Ota Kenji Uda Yoshio Araki Sho Okamoto Satoshi Maesawa Toshihiko Wakabayashi Atsushi Natsume Source Type: research

Insights into potential targeted nonsurgical therapies for the treatment of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E11. doi: 10.3171/2021.6.FOCUS21289.ABSTRACTSince its initial description in 1957 as an idiopathic disease, moyamoya disease has proved challenging to treat. Although the basic pathophysiology of this disease involves narrowing of the terminal carotid artery with compensatory angiogenesis, the molecular and cellular mechanisms underlying these changes are far more complex. In this article, the authors review the literature on the molecular and cellular pathophysiology of moyamoya disease with an emphasis on potential therapeutic targets.PMID:34469871 | DOI:10.3171/2021.6.FOCUS21289 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Dylan Goehner Sandeep Kandregula Christopher P Carroll Mario Zuccarello Bharat Guthikonda Jennifer A Kosty Source Type: research

Role of RNF213 polymorphism in defining quasi-moyamoya disease and definitive moyamoya disease
CONCLUSIONS: There are two forms of QMMD, one in which the vascular abnormality is associated with an underlying disease, and the other in which MMD is coincidentally complicated by an unrelated underlying disease. It has been suggested that the presence or absence of the RNF213 c.14576G>A mutation may be useful in distinguishing between these disease types.PMID:34469872 | DOI:10.3171/2021.5.FOCUS21182 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Eitaro Ishisaka Atsushi Watanabe Yasuo Murai Kazutaka Shirokane Fumihiro Matano Atsushi Tsukiyama Eiichi Baba Shunsuke Nakagawa Tomonori Tamaki Takayuki Mizunari Rokuya Tanikawa Akio Morita Source Type: research

Letter to the Editor. Digitalizing neurosurgical pedagogy
Neurosurg Focus. 2021 Sep;51(3):E12. doi: 10.3171/2021.5.FOCUS21292.NO ABSTRACTPMID:34469873 | DOI:10.3171/2021.5.FOCUS21292 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Chidinma M Wilson Nolan J Brown Donald K E Detchou Source Type: research

The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review
CONCLUSIONS: Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.PMID:34469862 | DOI:10.3171/2021.6.FOCUS21281 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Bhanu Jayanand Sudhir Arun Gowda Keelara Easwer Harihara Venkat Ken Kazumata Ananthalakshmy Sundararaman Source Type: research

Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E10. doi: 10.3171/2021.6.FOCUS21374.NO ABSTRACTPMID:34469863 | DOI:10.3171/2021.6.FOCUS21374 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Source Type: research

Introduction. Translational research advances in the evaluation and management of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E1. doi: 10.3171/2021.6.FOCUS21373.NO ABSTRACTPMID:34469864 | DOI:10.3171/2021.6.FOCUS21373 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Giuseppe Lanzino Gary K Steinberg Bin Xu Source Type: research

Experimental animal models for moyamoya disease and treatment: a pathogenesis-oriented scoping review
CONCLUSIONS: Models generated for MMD have involved three general approaches: surgical, immunological, and genetic. Although each reflects a key aspect of MMD pathogenesis, the failure of any individual model to recapitulate the development, progression, and consequences of the disease underscores the importance of future work in developing a multietiology model.PMID:34469865 | DOI:10.3171/2021.6.FOCUS21284 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Michael S Rallo Omar Akel Akhilesh Gurram Hai Sun Source Type: research

Experimental animal models for the study of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E4. doi: 10.3171/2021.5.FOCUS21282.ABSTRACTMoyamoya disease is a rare disorder of the cerebrovascular system affecting individuals in a bimodal age distribution and is characterized by progressive vascular stenosis of the bilateral supraclinoid internal carotid arteries with compensatory formation of collateral vessels at the base of the brain. Despite the disease's initial description in the literature in 1957, little progress has been made in the development of medical and surgical therapeutics due to, in no small part, the lack of effective experimental animal models. Currently, there is ...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Vijay Letchuman Leonel Ampie Panagiotis Mastorakos Daniel M S Raper Ryan T Kellogg Min S Park Source Type: research

The role of atorvastatin in collateral circulation formation induced by encephaloduroarteriosynangiosis: a prospective trial
CONCLUSIONS: The results of this prospective clinical trial have indicated that atorvastatin administered at 20 mg daily is safe and effective for the formation of postoperative collateral induced by EDAS.PMID:34469867 | DOI:10.3171/2021.6.FOCUS21112 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Qian-Nan Wang Xiang-Yang Bao Zheng-Xing Zou Xiao-Peng Wang Qian Zhang De-Sheng Li Ya-Qun Zhao Lian Duan Source Type: research

Motor excitability in bilateral moyamoya vasculopathy and the impact of revascularization
CONCLUSIONS: The study results suggested that, in the case of a bilateral chronic ischemia, a compensation mechanism between both hemispheres seemed to exist that normalized after revascularization surgery. A potential role of nTMS in predicting the efficacy of revascularization must be further assessed.PMID:34469868 | DOI:10.3171/2021.6.FOCUS21280 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Gueliz Acker Davide Giampiccolo Kerstin Rubarth Robert Mertens Anna Zdunczyk Juliane Hardt Daniel Jussen Heike Schneider Tizian Rosenstock Vera Mueller Thomas Picht Peter Vajkoczy Source Type: research

Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya
Neurosurg Focus. 2021 Sep;51(3):E8. doi: 10.3171/2021.6.FOCUS21283.ABSTRACTOBJECTIVE: Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya.METHODS: A retrospective surgical...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Joseph H Garcia Ramin A Morshed Ethan A Winkler Yi Li Christine K Fox Heather J Fullerton Caleb Rutledge Angad S Beniwal Michael T Lawton Adib A Abla Nalin Gupta Steven W Hetts Source Type: research

Transcriptome-wide analysis of intracranial artery in patients with moyamoya disease showing upregulation of immune response, and downregulation of oxidative phosphorylation and DNA repair
CONCLUSIONS: Transcriptome-wide analysis showed upregulation of genes for immune responses and downregulation of genes for DNA repair and oxidative phosphorylation within the intracranial artery of patients with MMD. These findings may represent clues to the pathophysiology of MMD.PMID:34469870 | DOI:10.3171/2021.6.FOCUS20870 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Fumiaki Kanamori Kinya Yokoyama Akinobu Ota Kazuhiro Yoshikawa Sivasundaram Karnan Mikio Maruwaka Kenzo Shimizu Shinji Ota Kenji Uda Yoshio Araki Sho Okamoto Satoshi Maesawa Toshihiko Wakabayashi Atsushi Natsume Source Type: research

Insights into potential targeted nonsurgical therapies for the treatment of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E11. doi: 10.3171/2021.6.FOCUS21289.ABSTRACTSince its initial description in 1957 as an idiopathic disease, moyamoya disease has proved challenging to treat. Although the basic pathophysiology of this disease involves narrowing of the terminal carotid artery with compensatory angiogenesis, the molecular and cellular mechanisms underlying these changes are far more complex. In this article, the authors review the literature on the molecular and cellular pathophysiology of moyamoya disease with an emphasis on potential therapeutic targets.PMID:34469871 | DOI:10.3171/2021.6.FOCUS21289 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Dylan Goehner Sandeep Kandregula Christopher P Carroll Mario Zuccarello Bharat Guthikonda Jennifer A Kosty Source Type: research

Role of RNF213 polymorphism in defining quasi-moyamoya disease and definitive moyamoya disease
CONCLUSIONS: There are two forms of QMMD, one in which the vascular abnormality is associated with an underlying disease, and the other in which MMD is coincidentally complicated by an unrelated underlying disease. It has been suggested that the presence or absence of the RNF213 c.14576G>A mutation may be useful in distinguishing between these disease types.PMID:34469872 | DOI:10.3171/2021.5.FOCUS21182 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Eitaro Ishisaka Atsushi Watanabe Yasuo Murai Kazutaka Shirokane Fumihiro Matano Atsushi Tsukiyama Eiichi Baba Shunsuke Nakagawa Tomonori Tamaki Takayuki Mizunari Rokuya Tanikawa Akio Morita Source Type: research

Letter to the Editor. Digitalizing neurosurgical pedagogy
Neurosurg Focus. 2021 Sep;51(3):E12. doi: 10.3171/2021.5.FOCUS21292.NO ABSTRACTPMID:34469873 | DOI:10.3171/2021.5.FOCUS21292 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Chidinma M Wilson Nolan J Brown Donald K E Detchou Source Type: research

The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review
CONCLUSIONS: Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.PMID:34469862 | DOI:10.3171/2021.6.FOCUS21281 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Bhanu Jayanand Sudhir Arun Gowda Keelara Easwer Harihara Venkat Ken Kazumata Ananthalakshmy Sundararaman Source Type: research

Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E10. doi: 10.3171/2021.6.FOCUS21374.NO ABSTRACTPMID:34469863 | DOI:10.3171/2021.6.FOCUS21374 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Source Type: research

Introduction. Translational research advances in the evaluation and management of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E1. doi: 10.3171/2021.6.FOCUS21373.NO ABSTRACTPMID:34469864 | DOI:10.3171/2021.6.FOCUS21373 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Giuseppe Lanzino Gary K Steinberg Bin Xu Source Type: research

Experimental animal models for moyamoya disease and treatment: a pathogenesis-oriented scoping review
CONCLUSIONS: Models generated for MMD have involved three general approaches: surgical, immunological, and genetic. Although each reflects a key aspect of MMD pathogenesis, the failure of any individual model to recapitulate the development, progression, and consequences of the disease underscores the importance of future work in developing a multietiology model.PMID:34469865 | DOI:10.3171/2021.6.FOCUS21284 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Michael S Rallo Omar Akel Akhilesh Gurram Hai Sun Source Type: research

Experimental animal models for the study of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E4. doi: 10.3171/2021.5.FOCUS21282.ABSTRACTMoyamoya disease is a rare disorder of the cerebrovascular system affecting individuals in a bimodal age distribution and is characterized by progressive vascular stenosis of the bilateral supraclinoid internal carotid arteries with compensatory formation of collateral vessels at the base of the brain. Despite the disease's initial description in the literature in 1957, little progress has been made in the development of medical and surgical therapeutics due to, in no small part, the lack of effective experimental animal models. Currently, there is ...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Vijay Letchuman Leonel Ampie Panagiotis Mastorakos Daniel M S Raper Ryan T Kellogg Min S Park Source Type: research

The role of atorvastatin in collateral circulation formation induced by encephaloduroarteriosynangiosis: a prospective trial
CONCLUSIONS: The results of this prospective clinical trial have indicated that atorvastatin administered at 20 mg daily is safe and effective for the formation of postoperative collateral induced by EDAS.PMID:34469867 | DOI:10.3171/2021.6.FOCUS21112 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Qian-Nan Wang Xiang-Yang Bao Zheng-Xing Zou Xiao-Peng Wang Qian Zhang De-Sheng Li Ya-Qun Zhao Lian Duan Source Type: research

Motor excitability in bilateral moyamoya vasculopathy and the impact of revascularization
CONCLUSIONS: The study results suggested that, in the case of a bilateral chronic ischemia, a compensation mechanism between both hemispheres seemed to exist that normalized after revascularization surgery. A potential role of nTMS in predicting the efficacy of revascularization must be further assessed.PMID:34469868 | DOI:10.3171/2021.6.FOCUS21280 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Gueliz Acker Davide Giampiccolo Kerstin Rubarth Robert Mertens Anna Zdunczyk Juliane Hardt Daniel Jussen Heike Schneider Tizian Rosenstock Vera Mueller Thomas Picht Peter Vajkoczy Source Type: research

Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya
Neurosurg Focus. 2021 Sep;51(3):E8. doi: 10.3171/2021.6.FOCUS21283.ABSTRACTOBJECTIVE: Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya.METHODS: A retrospective surgical...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Joseph H Garcia Ramin A Morshed Ethan A Winkler Yi Li Christine K Fox Heather J Fullerton Caleb Rutledge Angad S Beniwal Michael T Lawton Adib A Abla Nalin Gupta Steven W Hetts Source Type: research

Transcriptome-wide analysis of intracranial artery in patients with moyamoya disease showing upregulation of immune response, and downregulation of oxidative phosphorylation and DNA repair
CONCLUSIONS: Transcriptome-wide analysis showed upregulation of genes for immune responses and downregulation of genes for DNA repair and oxidative phosphorylation within the intracranial artery of patients with MMD. These findings may represent clues to the pathophysiology of MMD.PMID:34469870 | DOI:10.3171/2021.6.FOCUS20870 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Fumiaki Kanamori Kinya Yokoyama Akinobu Ota Kazuhiro Yoshikawa Sivasundaram Karnan Mikio Maruwaka Kenzo Shimizu Shinji Ota Kenji Uda Yoshio Araki Sho Okamoto Satoshi Maesawa Toshihiko Wakabayashi Atsushi Natsume Source Type: research

Insights into potential targeted nonsurgical therapies for the treatment of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E11. doi: 10.3171/2021.6.FOCUS21289.ABSTRACTSince its initial description in 1957 as an idiopathic disease, moyamoya disease has proved challenging to treat. Although the basic pathophysiology of this disease involves narrowing of the terminal carotid artery with compensatory angiogenesis, the molecular and cellular mechanisms underlying these changes are far more complex. In this article, the authors review the literature on the molecular and cellular pathophysiology of moyamoya disease with an emphasis on potential therapeutic targets.PMID:34469871 | DOI:10.3171/2021.6.FOCUS21289 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Dylan Goehner Sandeep Kandregula Christopher P Carroll Mario Zuccarello Bharat Guthikonda Jennifer A Kosty Source Type: research

Role of RNF213 polymorphism in defining quasi-moyamoya disease and definitive moyamoya disease
CONCLUSIONS: There are two forms of QMMD, one in which the vascular abnormality is associated with an underlying disease, and the other in which MMD is coincidentally complicated by an unrelated underlying disease. It has been suggested that the presence or absence of the RNF213 c.14576G>A mutation may be useful in distinguishing between these disease types.PMID:34469872 | DOI:10.3171/2021.5.FOCUS21182 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Eitaro Ishisaka Atsushi Watanabe Yasuo Murai Kazutaka Shirokane Fumihiro Matano Atsushi Tsukiyama Eiichi Baba Shunsuke Nakagawa Tomonori Tamaki Takayuki Mizunari Rokuya Tanikawa Akio Morita Source Type: research

Letter to the Editor. Digitalizing neurosurgical pedagogy
Neurosurg Focus. 2021 Sep;51(3):E12. doi: 10.3171/2021.5.FOCUS21292.NO ABSTRACTPMID:34469873 | DOI:10.3171/2021.5.FOCUS21292 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Chidinma M Wilson Nolan J Brown Donald K E Detchou Source Type: research

The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review
CONCLUSIONS: Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.PMID:34469862 | DOI:10.3171/2021.6.FOCUS21281 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Bhanu Jayanand Sudhir Arun Gowda Keelara Easwer Harihara Venkat Ken Kazumata Ananthalakshmy Sundararaman Source Type: research

Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E10. doi: 10.3171/2021.6.FOCUS21374.NO ABSTRACTPMID:34469863 | DOI:10.3171/2021.6.FOCUS21374 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Source Type: research

Introduction. Translational research advances in the evaluation and management of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E1. doi: 10.3171/2021.6.FOCUS21373.NO ABSTRACTPMID:34469864 | DOI:10.3171/2021.6.FOCUS21373 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Giuseppe Lanzino Gary K Steinberg Bin Xu Source Type: research

Experimental animal models for moyamoya disease and treatment: a pathogenesis-oriented scoping review
CONCLUSIONS: Models generated for MMD have involved three general approaches: surgical, immunological, and genetic. Although each reflects a key aspect of MMD pathogenesis, the failure of any individual model to recapitulate the development, progression, and consequences of the disease underscores the importance of future work in developing a multietiology model.PMID:34469865 | DOI:10.3171/2021.6.FOCUS21284 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Michael S Rallo Omar Akel Akhilesh Gurram Hai Sun Source Type: research

Experimental animal models for the study of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E4. doi: 10.3171/2021.5.FOCUS21282.ABSTRACTMoyamoya disease is a rare disorder of the cerebrovascular system affecting individuals in a bimodal age distribution and is characterized by progressive vascular stenosis of the bilateral supraclinoid internal carotid arteries with compensatory formation of collateral vessels at the base of the brain. Despite the disease's initial description in the literature in 1957, little progress has been made in the development of medical and surgical therapeutics due to, in no small part, the lack of effective experimental animal models. Currently, there is ...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Vijay Letchuman Leonel Ampie Panagiotis Mastorakos Daniel M S Raper Ryan T Kellogg Min S Park Source Type: research

The role of atorvastatin in collateral circulation formation induced by encephaloduroarteriosynangiosis: a prospective trial
CONCLUSIONS: The results of this prospective clinical trial have indicated that atorvastatin administered at 20 mg daily is safe and effective for the formation of postoperative collateral induced by EDAS.PMID:34469867 | DOI:10.3171/2021.6.FOCUS21112 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Qian-Nan Wang Xiang-Yang Bao Zheng-Xing Zou Xiao-Peng Wang Qian Zhang De-Sheng Li Ya-Qun Zhao Lian Duan Source Type: research

Motor excitability in bilateral moyamoya vasculopathy and the impact of revascularization
CONCLUSIONS: The study results suggested that, in the case of a bilateral chronic ischemia, a compensation mechanism between both hemispheres seemed to exist that normalized after revascularization surgery. A potential role of nTMS in predicting the efficacy of revascularization must be further assessed.PMID:34469868 | DOI:10.3171/2021.6.FOCUS21280 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Gueliz Acker Davide Giampiccolo Kerstin Rubarth Robert Mertens Anna Zdunczyk Juliane Hardt Daniel Jussen Heike Schneider Tizian Rosenstock Vera Mueller Thomas Picht Peter Vajkoczy Source Type: research

Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya
Neurosurg Focus. 2021 Sep;51(3):E8. doi: 10.3171/2021.6.FOCUS21283.ABSTRACTOBJECTIVE: Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya.METHODS: A retrospective surgical...
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Joseph H Garcia Ramin A Morshed Ethan A Winkler Yi Li Christine K Fox Heather J Fullerton Caleb Rutledge Angad S Beniwal Michael T Lawton Adib A Abla Nalin Gupta Steven W Hetts Source Type: research

Transcriptome-wide analysis of intracranial artery in patients with moyamoya disease showing upregulation of immune response, and downregulation of oxidative phosphorylation and DNA repair
CONCLUSIONS: Transcriptome-wide analysis showed upregulation of genes for immune responses and downregulation of genes for DNA repair and oxidative phosphorylation within the intracranial artery of patients with MMD. These findings may represent clues to the pathophysiology of MMD.PMID:34469870 | DOI:10.3171/2021.6.FOCUS20870 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Fumiaki Kanamori Kinya Yokoyama Akinobu Ota Kazuhiro Yoshikawa Sivasundaram Karnan Mikio Maruwaka Kenzo Shimizu Shinji Ota Kenji Uda Yoshio Araki Sho Okamoto Satoshi Maesawa Toshihiko Wakabayashi Atsushi Natsume Source Type: research

Insights into potential targeted nonsurgical therapies for the treatment of moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E11. doi: 10.3171/2021.6.FOCUS21289.ABSTRACTSince its initial description in 1957 as an idiopathic disease, moyamoya disease has proved challenging to treat. Although the basic pathophysiology of this disease involves narrowing of the terminal carotid artery with compensatory angiogenesis, the molecular and cellular mechanisms underlying these changes are far more complex. In this article, the authors review the literature on the molecular and cellular pathophysiology of moyamoya disease with an emphasis on potential therapeutic targets.PMID:34469871 | DOI:10.3171/2021.6.FOCUS21289 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Dylan Goehner Sandeep Kandregula Christopher P Carroll Mario Zuccarello Bharat Guthikonda Jennifer A Kosty Source Type: research

Role of RNF213 polymorphism in defining quasi-moyamoya disease and definitive moyamoya disease
CONCLUSIONS: There are two forms of QMMD, one in which the vascular abnormality is associated with an underlying disease, and the other in which MMD is coincidentally complicated by an unrelated underlying disease. It has been suggested that the presence or absence of the RNF213 c.14576G>A mutation may be useful in distinguishing between these disease types.PMID:34469872 | DOI:10.3171/2021.5.FOCUS21182 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Eitaro Ishisaka Atsushi Watanabe Yasuo Murai Kazutaka Shirokane Fumihiro Matano Atsushi Tsukiyama Eiichi Baba Shunsuke Nakagawa Tomonori Tamaki Takayuki Mizunari Rokuya Tanikawa Akio Morita Source Type: research

Letter to the Editor. Digitalizing neurosurgical pedagogy
Neurosurg Focus. 2021 Sep;51(3):E12. doi: 10.3171/2021.5.FOCUS21292.NO ABSTRACTPMID:34469873 | DOI:10.3171/2021.5.FOCUS21292 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Chidinma M Wilson Nolan J Brown Donald K E Detchou Source Type: research

The mechanobiological theory: a unifying hypothesis on the pathogenesis of moyamoya disease based on a systematic review
CONCLUSIONS: Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.PMID:34469862 | DOI:10.3171/2021.6.FOCUS21281 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Bhanu Jayanand Sudhir Arun Gowda Keelara Easwer Harihara Venkat Ken Kazumata Ananthalakshmy Sundararaman Source Type: research

Editorial. Growing research: how neurosurgeons can lead the development of nonsurgical treatments for moyamoya disease
Neurosurg Focus. 2021 Sep;51(3):E10. doi: 10.3171/2021.6.FOCUS21374.NO ABSTRACTPMID:34469863 | DOI:10.3171/2021.6.FOCUS21374 (Source: Neurosurgical Focus)
Source: Neurosurgical Focus - September 1, 2021 Category: Neurosurgery Authors: Edward R Smith Source Type: research