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No Shinkei Geka. Neurological Surgery[JOURNAL]

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[History and Current Situation of Indirect Bypass Surgery for Moyamoya Disease].

Hishikawa T

No Shinkei Geka · 2025 May · PMID 40438013 · Publisher ↗

In indirect bypass surgery for moyamoya disease(MMD) angiogenesis is induced by attaching extracranial tissues to the brain surface, and the external carotid artery system supplements hemodynamic insufficiency in the int... In indirect bypass surgery for moyamoya disease(MMD) angiogenesis is induced by attaching extracranial tissues to the brain surface, and the external carotid artery system supplements hemodynamic insufficiency in the internal carotid artery system. This procedure is specific to patients with MMD. The history of this procedure includes the development of blood supply sources and ways to efficiently supply blood to various areas of the brain. The mechanism underlying the angiogenesis induced by this procedure remains unclear. Evaluation of the indications for this procedure in adult patients with ischemic MMD and elucidation of its preventive effect on hemorrhagic MMD are warranted.

[Association of Rare Variants and Moyamoya Disease].

Akagawa H

No Shinkei Geka · 2025 May · PMID 40438012 · Publisher ↗

Rare variants other than p.R4810K(rs112735431) have been identified in Asian and European patients with moyamoya disease. Several studies have consistently demonstrated that putative functional variants are significantl... Rare variants other than p.R4810K(rs112735431) have been identified in Asian and European patients with moyamoya disease. Several studies have consistently demonstrated that putative functional variants are significantly more prevalent in patients than in the general population, with the aid of bioinformatics tools, such as Combined Annotation-Dependent Depletion. Among these rare susceptibility variants, p.R4062Q(rs1555676035) has been repeatedly reported in severe pediatric cases with moyamoya disease. Three-dimensional structural analysis suggested that this may cause a loss of polar contact with the D4003 residue, leading to instability of the E3 ligase module in RNF213. Rare susceptibility variants tend to accumulate in this E3 module in pediatric cases, which may influence the severity of the clinical manifestations. Further research, including in vitro and in vivo functional analyses of the variants, is required to develop precision medicine.

[Pathophysiology of -related Vasculopathy].

Ihara M

No Shinkei Geka · 2025 May · PMID 40438011 · Publisher ↗

The p.R4810K variant of the gene was identified as the founder variant of East Asian moyamoya disease. The association of the p.R4810K variant with non-moyamoya intracranial arterial stenosis has been demonstrated, and... The p.R4810K variant of the gene was identified as the founder variant of East Asian moyamoya disease. The association of the p.R4810K variant with non-moyamoya intracranial arterial stenosis has been demonstrated, and the concept of -related vasculopathy has been proposed, suggesting a continuous spectrum of moyamoya disease. Additionally, moyamoya disease is occasionally accompanied by polyvascular disease involving intracranial and neck vessels, coronary arteries(especially vasospastic angina), pulmonary arteries, aorta, abdominal visceral arteries, and peripheral arteries, which are associated with variants, mainly p.R4810K. The severity of vascular diseases caused by the variant is inconsistent, and some environmental and genetic factors are believed to jointly define the phenotype. variants confer the greatest risk of cardiovascular diseases in East Asia. Therefore, successful targeting of these variants is essential for controlling cardiovascular diseases, including stroke, in East Asian countries.

[Moyamoya Disease and the Gene].

Miyawaki S

No Shinkei Geka · 2025 May · PMID 40438010 · Publisher ↗

Genetic research on moyamoya disease(MMD) has advanced significantly following the identification of as a susceptibility gene. Approximately 80% of Japanese patients with MMD harbor the p.Arg4810Lys variant, which has... Genetic research on moyamoya disease(MMD) has advanced significantly following the identification of as a susceptibility gene. Approximately 80% of Japanese patients with MMD harbor the p.Arg4810Lys variant, which has been increasingly linked to variations in clinical phenotypes, including disease progression, mode of onset, and postoperative outcomes. Of note, this variant is also associated with intracranial arterial stenosis that does not meet the diagnostic criteria for MMD, as well as with systemic vascular conditions such as non-cardioembolic ischemic stroke, coronary artery disease, and pulmonary hypertension. Despite its strong association with the disease, the p.Arg4810Lys variant is present in approximately 2% of the general population, suggesting incomplete penetrance and the involvement of additional pathogenic factors. In parallel, researchers have examined the clinical relevance of variants other than p.Arg4810Lys and have identified rare mutations that may contribute to disease severity. Moreover, large-scale genetic analyses have identified additional susceptibility genes, such as DIAPH1 and ANO1, whose roles in MMD pathogenesis remain under investigation. Although substantial advances have been made in elucidating the genetic architecture of MMD, the precise mechanisms underlying disease onset remain elusive and represent an important area of ongoing research.

[Brief History of the Search for Susceptibility Loci for Moyamoya Disease].

Tada M

No Shinkei Geka · 2025 May · PMID 40438009 · Publisher ↗

Here, I briefly describe the determination of locus 17q25 on chromosome 17 for a moyamoya disease susceptibility gene, focusing on our study. At the beginning of the study, linkage analysis was challenging. However, our... Here, I briefly describe the determination of locus 17q25 on chromosome 17 for a moyamoya disease susceptibility gene, focusing on our study. At the beginning of the study, linkage analysis was challenging. However, our efforts finally achieved statistically significant results at locus 17q25 with a logarithm of odds(LOD) score of 3.11, a maximum LOD score of 4.58, and p-value of 0.00001 with parametric linkage analysis, multipoint analysis, and nonparametric Affected Pedigree Member analysis, respectively. This was due to the good fortune of choosing chromosome 17 as the primary target, given that neurofibromatosis type 1 and moyamoya disease occur simultaneously in some cases. Moreover, our success was largely due to the contributions of our collaborators who precisely determined the disease traits and collected DNA(leukocyte) samples from 103 individuals from 24 families. After our publication in 2000, a research group from Kyoto University searched for the locus 17q25.3 and discovered a mutation in gene. Simultaneously, a group from Tohoku University performed a genome-wide association study and determined to be a susceptibility gene for moyamoya disease. This occurred 11 years after our first results.

[Severity Classification of Moyamoya Disease].

Takahashi JC

No Shinkei Geka · 2025 May · PMID 40438008 · Publisher ↗

Moyamoya disease is currently classified as one of the "Specified Intractable Diseases" by the Ministry of Health, Labor, and Welfare. Since the establishment of this classification in 2014, individuals with mild or nonp... Moyamoya disease is currently classified as one of the "Specified Intractable Diseases" by the Ministry of Health, Labor, and Welfare. Since the establishment of this classification in 2014, individuals with mild or nonpersistent symptoms or patients who underwent bypass surgery more than 5 years ago are no longer eligible for certification. To address this issue, the Moyamoya Disease Research Group, under the Ministry of Health, Labor, and Welfare, initiated a revision of the severity criteria in 2021; official revision was passed in 2024. The main change in the revision is the "shift from focusing on fixed symptoms to prioritizing future medical needs."

[Diagnosis and Management Guidelines for Moyamoya Disease].

Fujimura M

No Shinkei Geka · 2025 May · PMID 40438007 · Publisher ↗

Moyamoya disease(MMD) is a rare cerebrovascular disease characterized by progressive stenosis of the internal carotid artery terminus and an abnormal formation of a vascular network at the base of the brain. Superficial... Moyamoya disease(MMD) is a rare cerebrovascular disease characterized by progressive stenosis of the internal carotid artery terminus and an abnormal formation of a vascular network at the base of the brain. Superficial temporal artery-middle cerebral artery(STA-MCA) bypass, either as a direct or combined revascularization procedure, is a reasonable management choice for patients with symptomatic MMD. STA-MCA bypass prevents cerebral ischemic attacks by improving cerebral blood flow. Recent evidence further suggests that direct revascularization reduces the potential risk of rebleeding in patients with MMD with posterior hemorrhage who have an extremely high annual rebleeding rate. Despite the favorable long-term outcomes of STA-MCA bypass, cerebral hyperperfusion syndrome is a potential complication of this procedure that can result in focal neurologic deficits and/or delayed intracerebral hemorrhage. Therefore, recent guideline recommendations indicate that STA-MCA bypass is a reasonable choice for symptomatic patients with MMD, together with intensive perioperative care for blood pressure control.

[Historical Review and Perspective on the Diagnostic Criteria of Moyamoya Disease].

Kuroda S, Miyamoto S

No Shinkei Geka · 2025 May · PMID 40438006 · Publisher ↗

We reviewed the historical aspects of the diagnostic criteria for moyamoya disease since 1978. Based on novel knowledge of moyamoya disease, the diagnostic criteria have been revised in 1988, 1995, 2009, 2015, and 2021.... We reviewed the historical aspects of the diagnostic criteria for moyamoya disease since 1978. Based on novel knowledge of moyamoya disease, the diagnostic criteria have been revised in 1988, 1995, 2009, 2015, and 2021. The most recent diagnostic criteria emphasize the importance of arterial shrinkage, which can be observed in moyamoya disease, and have also reorganized the critical comorbid disorders of quasi-moyamoya disease(moyamoya syndrome). We also discussed the future perspectives on the diagnosis of moyamoya disease.

[History and Recent Advancements of the Research Committee on Moyamoya Disease of the Ministry of Health, Labor and Welfare, Japan].

Miyamoto S, Kuroda S

No Shinkei Geka · 2025 May · PMID 40438005 · Publisher ↗

The Research Committee on Moyamoya Disease(Spontaneous Occlusion of the Circle of Willis) of the Ministry of Health, Labor and Welfare of Japan was established in 1977. The committee has developed diagnostic criteria and... The Research Committee on Moyamoya Disease(Spontaneous Occlusion of the Circle of Willis) of the Ministry of Health, Labor and Welfare of Japan was established in 1977. The committee has developed diagnostic criteria and therapeutic guidelines for moyamoya disease. Several multicenter clinical studies, conducted by the research committee, have clarified pathophysiological features of the disease and provided strong evidence for effective treatments.

[The 2040 Problem that Neurosurgeons Should Know].

Muramatsu K, Matsuda S

No Shinkei Geka · 2025 Mar · PMID 40155346 · Publisher ↗

Japan's healthcare system is undergoing significant transformations, driven by demographic shifts, with critical challenges anticipated in 2025 and 2040. The Ministry of Health, Labor, and Welfare is promoting regional m... Japan's healthcare system is undergoing significant transformations, driven by demographic shifts, with critical challenges anticipated in 2025 and 2040. The Ministry of Health, Labor, and Welfare is promoting regional medical care vision plans to restructure healthcare delivery across different administrative levels, addressing evolving medical demands and population changes. However, the implementation of these plans has been uneven, with many regions focusing primarily on reducing hospital beds rather than on effectively redistributing medical roles and functions. Time constraints have hindered meaningful discussions regarding comprehensive healthcare restructuring. The text illustrates these challenges through two case studies in the Fukuoka Prefecture: Fukuoka-Itoshima and Keichiku areas. These regions demonstrate contrasting demographic trajectories with significantly aging and declining working-age populations. Notably, the Fukuoka-Itoshima area anticipates a substantial increase in cerebrovascular disease hospitalizations, whereas Keichiku expects moderate changes. This analysis suggests that future neurosurgeons must be adaptable professionals capable of navigating clinical, management, and policy domains. By 2040, they should proactively develop strategies to address demographic changes and involve current medical trainees and students in strategic planning of the healthcare landscape.

[Prehospital Stroke Rescue and Acute Stroke Care:Focusing on Acute Large Vessel Occlusion].

Ohta T

No Shinkei Geka · 2025 Mar · PMID 40155345 · Publisher ↗

The implementation of standardized prehospital stroke assessments, transport decision-making, and treatment is crucial for optimizing acute stroke care. This includes the efficient utilization of intravenous thrombolysis... The implementation of standardized prehospital stroke assessments, transport decision-making, and treatment is crucial for optimizing acute stroke care. This includes the efficient utilization of intravenous thrombolysis for acute ischemic stroke and mechanical thrombectomy for acute large-vessel occlusion (LVO). In the existing acute stroke care system, it is imperative to promptly recognize a stroke, gather essential information, select an appropriate hospital with the necessary capabilities, and establish a rapid transport pathway. The Japan Stroke Association has designated primary stroke centers for intravenous thrombolysis and mechanical thrombectomy, making this information accessible to both the general public and healthcare professionals. Additionally, the JSS/JAAM standard LVO Scale, comprising six items, could be the preferred scale for predicting LVO in Japan, with efforts underway to address various challenges associated with its clinical application.

[Using Futuristic Advanced Operating Rooms].

Muragaki Y, Kawamata T, Masamune K … +1 more , Sasayama T

No Shinkei Geka · 2025 Mar · PMID 40155344 · Publisher ↗

Future operating rooms (ORs) should integrate cutting-edge technologies to address the limitations of traditional surgical environments. Leveraging intraoperative imaging, the Internet of Things, and artificial intellige... Future operating rooms (ORs) should integrate cutting-edge technologies to address the limitations of traditional surgical environments. Leveraging intraoperative imaging, the Internet of Things, and artificial intelligence enables real-time data integration, precise decision-making, and improved surgical outcomes. This approach transforms implicit knowledge into structured and actionable insights, thereby enhancing efficiency. Technologies, such as intraoperative magnetic resonance imaging, hybrid ORs, and surgical robots offer advanced capabilities, reduce complications, and standardize surgical performance. Remote surgery and telemonitoring extend expert care to underserved regions, addressing geographical disparities. However, challenges such as high implementation costs, data security, and standardization, persist. International efforts, such as OPeLiNK, aim to standardize data protocols and interoperability. Future ORs are envisioned as dynamic and intelligent environments that can adapt to complex needs, ensuring enhanced patient safety, clinical outcomes, and equitable healthcare delivery.

[Effective Use of Evolutionary Operating Room].

Motomura K, Saito R

No Shinkei Geka · 2025 Mar · PMID 40155343 · Publisher ↗

Brain parenchyma tumors, typically gliomas, often arise in eloquent areas and complex regions deep within the brain that are involved in language, motor, and higher cognitive functions. Brain tumor surgery aims to safely... Brain parenchyma tumors, typically gliomas, often arise in eloquent areas and complex regions deep within the brain that are involved in language, motor, and higher cognitive functions. Brain tumor surgery aims to safely remove as much of the tumor as possible while preserving brain function. However, invasive brain tumors, such as gliomas, are often difficult to distinguish from the normal brain, and removal of the entire tumor may inadvertently damage the normal brain tissue, resulting in the risk of serious postoperative complications. Additionally, brain tissue is subject to brain deformation, called brain shift, because of cerebrospinal fluid drainage and tumor removal. This reduces the accuracy of neuronavigation during preoperative planning, resulting in differences in the exact locations of the tumor and nerve fibers. Intraoperative magnetic resonance imaging (MRI) enabled to identify the exact tumor position and residual tumor in real-time perioperatively, compared to surgery that relies on the surgeon's experience and skill, enabling highly accurate surgery. Moreover, the fusion and comparison of preoperative images and latest intraoperative MRI images enable the accurate evaluation of tumor location and safe tumor removal. Intraoperative MRI imaging-assisted techniques that can capture residual tumor morphology and awake surgery that preserves brain function should be integrated to complement each other's roles.

[Application of AR/VR Technologies in Neurosurgery].

Maruyama K

No Shinkei Geka · 2025 Mar · PMID 40155342 · Publisher ↗

Augmented reality (AR) and virtual reality (VR) technologies have been rapidly developing and are widely used. They have also been applied in the field of neurosurgery through the use of various devices. Integration with... Augmented reality (AR) and virtual reality (VR) technologies have been rapidly developing and are widely used. They have also been applied in the field of neurosurgery through the use of various devices. Integration with microsurgery, initially introduced as an extension of surgical navigation, may be the most widespread AR technology at present. Tablet devices, smart glasses, and 3D printers can all be used for surgical assistance or training. Although these devices still have some issues associated with imaging, registration error, brain shift, costs, and human resources, they are expected to improve the accuracy and safety of neurosurgery.

[Intraoperative Utilization of Preoperative Virtual Reality Surgical Simulation].

Kin T

No Shinkei Geka · 2025 Mar · PMID 40155341 · Publisher ↗

The use of preoperative virtual reality simulations based on medical imaging for surgical support can be divided into two phases; preoperative and intraoperative. In the preoperative phase, fusion three-dimensional (3D)... The use of preoperative virtual reality simulations based on medical imaging for surgical support can be divided into two phases; preoperative and intraoperative. In the preoperative phase, fusion three-dimensional (3D) images constructed from multiple medical imaging datasets are employed to observe the 3D anatomical structures around the lesion and surgical approach in detail. Based on the anatomical findings obtained from fusion 3D images, surgical planning is completed by virtually simulating surgical procedures, such as craniotomy, tissue deformation, and using surgical instruments. In the intraoperative phase, a surgical plan was used. These include tools for displaying surgical plans on intraoperative navigation systems, viewing fusion 3D images on tablet computers, and combining fusion 3D images with the surgical field using mixed-reality technology. This section focuses on preoperative virtual reality simulation and the intraoperative application of 3D image fusion, highlighting efforts at our institution through presentations of illustrative cases.

[Practical Applications of Robot Systems in Epilepsy Surgery].

Kagawa K, Horie N, Iida K

No Shinkei Geka · 2025 Mar · PMID 40155340 · Publisher ↗

Subdural electrode (SDE) implantation and stereotactic electroencephalography (SEEG) represent two primary invasive monitoring techniques employed in epilepsy surgery. In North America, the advent of commercially availab... Subdural electrode (SDE) implantation and stereotactic electroencephalography (SEEG) represent two primary invasive monitoring techniques employed in epilepsy surgery. In North America, the advent of commercially available surgical robotic systems has initiated a paradigm shift from SDE to SEEG implantation. Advances in robotic technology have enabled the precise and efficient placement of depth electrodes for SEEG. In Japan, robot-assisted stereotactic electrode placement has been covered by National Health Insurance since 2020, further promoting its adoption. SEEG relies exclusively on intracerebral depth electrodes, which are stereographically inserted through twist drill holes or burr holes, eliminating the need for craniotomy-a requirement for SDE implantation. The planning of electrode trajectories is critical and must be meticulously performed using three-dimensional gadolinium-enhanced magnetic resonance imaging datasets to avoid vascular structures. Unlike SDE, SEEG allows for accurate sampling of cortical areas at the surface of hemispheres and bottom of sulci and deep-seated structures, such as the insular cortex, cingulate gyrus, and medial temporal lobes. This section provides a comprehensive overview of the indications for SEEG, the method of electrode implantation using robotic systems, the advantages of SEEG over other monitoring techniques, and its associated risks.

[Utilization of Intraoperative Navigation in Stereotactic Neurosurgery].

Fukuda M, Ota T

No Shinkei Geka · 2025 Mar · PMID 40155339 · Publisher ↗

Recently, a navigation software has been introduced in the field of stereotactic neurosurgery. BrainLab Elements (BrainLab Corp.) has several functions in image analysis, such as image fusion, auto-segmentation, software... Recently, a navigation software has been introduced in the field of stereotactic neurosurgery. BrainLab Elements (BrainLab Corp.) has several functions in image analysis, such as image fusion, auto-segmentation, software for stereotactic surgery, and lead localization. In preparation for deep brain stimulation (DBS) electrode implantation, many sequences of magnetic resonance (MR) and computed tomography (CT) images are imported into the elements and fused automatically. Subsequently, important deep structures, such as the subthalamic nucleus (STN) , thalamus, and globus pallidus, were automatically segmented. This segmentation provides information on the outline of those that are invisible based on normal MR equipment and helps set the tentative target. Postoperatively, the DBS electrode data were extracted from postoperative CT images and fused with preoperative MR images. Using Stimview XT software (Boston Scientific Corp.) installed in the stimulation programmer, we confirmed the anatomical relationship between the location of the electrodes and structures of the stimulation target and adjusted the stimulation conditions. In robot-assisted DBS electrode implantation, elements could be useful in setting the tentative target and frame-based stereotactic surgery. Navigation systems are useful in the field of stereotactic neurosurgery and can be further developed eventually.

[Real-Time Artificial Intelligence Assistance in Neuroendovascular Surgery].

Kono K

No Shinkei Geka · 2025 Mar · PMID 40155338 · Publisher ↗

In neuroendovascular surgery, operators must manipulate devices, such as microcatheters and guidewires, with millimeter precision while simultaneously monitoring multiple areas across up to four radiography monitors. Alt... In neuroendovascular surgery, operators must manipulate devices, such as microcatheters and guidewires, with millimeter precision while simultaneously monitoring multiple areas across up to four radiography monitors. Although both operators and assistants maintain careful observation, any oversight can lead to serious complications. To address this challenge, we have developed a real-time assistance system using artificial intelligence (AI). This paper discusses the practical experience, current challenges, and future prospects of the intraoperative real-time AI assistance system implemented in Japan.

[Use of Fluid Imaging in Endovascular Neurotherapy].

Suzuki T

No Shinkei Geka · 2025 Mar · PMID 40155337 · Publisher ↗

Recently, neuroendovascular surgery has increased; however, it is impossible to observe aneurysm wall conditions, such as thinning and rupture sites, unlike neck clipping. Computational fluid dynamics (CFD) is a useful t... Recently, neuroendovascular surgery has increased; however, it is impossible to observe aneurysm wall conditions, such as thinning and rupture sites, unlike neck clipping. Computational fluid dynamics (CFD) is a useful tool in endovascular surgery for predicting danger zones in cerebral aneurysms. Moreover, its risk of recurrence is challenging compared with open surgery, especially coil compaction, leading to recanalization and the risk of aneurysm rupture. De novo neck formation in coiled aneurysms has been reported over a long follow-up period. Abnormal hemodynamic stress is associated with the recurrence of treated aneurysms. Here, we introduce CFD analysis as a useful tool for neurosurgeons performing hemodynamic imaging in representative cases.

[The Utility of Hybrid Operating Rooms for the Treatment of Cerebrovascular Diseases].

Kan I, Kato N, Nagayama G … +5 more , Sano T, Fuga M, Hataoka S, Ishibashi T, Murayama Y

No Shinkei Geka · 2025 Mar · PMID 40155336 · Publisher ↗

Endovascular neurosurgery has been established as a first-line therapy for cerebrovascular diseases. Although many endovascular procedures can be performed in conventional angiosuites, a sterile and well-prepared environ... Endovascular neurosurgery has been established as a first-line therapy for cerebrovascular diseases. Although many endovascular procedures can be performed in conventional angiosuites, a sterile and well-prepared environment is needed to perform combined surgical exposure and endovascular procedures for complex neurovascular diseases, such as thrombosed giant aneurysms. Therefore, we established a hybrid operating room (OR) to treat cerebrovascular disease. The first hybrid OR was established in 2003 using a biplane DSA system with a surgical OR table system. A new concept involving the implementation of industrial robot technology in angiography systems was developed in collaboration with Siemens, and a new image-guided surgery environment was established (Robotic DSA). Our hospital currently has three hybrid operating rooms (one with a biplane angiography machine and two with a robotic DSA). This study describes the utilization of hybrid operating rooms for treating cerebrovascular diseases and their disadvantages.
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