{"title":"Genetic Basis and Clinical Management of Schwannomatosis.","authors":"Shohei Nagasaka, Ji Hoon Phi","doi":"10.3340/jkns.2025.0001","DOIUrl":"10.3340/jkns.2025.0001","url":null,"abstract":"<p><p>Schwannomatosis (SWN) is now recognized as a broad classification that includes neurofibromatosis (NF) type 2, reflecting their shared genetic and phenotypic characteristics. Previously, SWN and NF type 2 were considered distinct clinical entities; however, the 2022 classification revision has unified them under the umbrella of SWN, with NF type 2 now referred to as NF2-related SWN. SWN arises from mutations in NF2, SMARCB1 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1) or LZTR1 (leucine zipper like transcription regulator 1). Recent diagnostic criteria for SWN incorporate molecular classification, including \"NF2-related SWN\", \"SMARCB1-related SWN\", \"LZTR1-related SWN\", \"22q-related SWN\", \"SWN-not otherwise specified\", or \"SWN-not elsewhere classified\". NF2-related SWN is a genetic condition where all individuals with a germline or constitutional NF2 mutation are destined to develop the disease. The pathogenesis of SMARCB1- or LZTR1-related SWN follows a three-step, four-hit model. This involves retention of the mutated germline SMARCB1 or LZTR1 allele in the tumor, loss of the wild-type chromosome 22, and somatic mutation in the NF2 gene. Clinically, NF2-related SWN involves bilateral vestibular schwannomas, with treatment options including microsurgery, radiotherapy, and bevacizumab, each with specific benefits and limitations. Patients with SWN frequently present with chronic pain caused by schwannomas, which often does not correlate with tumor size, location, or burden. Management of SWN is primarily symptom-based. Surgical intervention is reserved for symptomatic lesions, particularly in cases of spinal cord compression or significant functional impairments. Multidisciplinary approaches to pain management are critical for enhancing quality of life. Although malignant transformation of schwannomas is a potential risk, the life expectancy of individuals with SWN is nearly normal. Despite advancements in understanding SWN, further research is necessary to elucidate the underlying genetic mechanisms and to develop targeted therapeutic strategies for this complex disorder.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":"286-293"},"PeriodicalIF":1.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12062529/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143572920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Preface : Invited Issue Editor, Professor Tai-Tong Wong and the Cancer Predisposition Syndrome.","authors":"Ji Hoon Phi, Tai-Tong Wong, Seung-Ki Kim","doi":"10.3340/jkns.2025.0063","DOIUrl":"10.3340/jkns.2025.0063","url":null,"abstract":"","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":"249-251"},"PeriodicalIF":1.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12062533/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143779952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Minho Yang, Gung Ju Kim, Juhee Jeon, Yukyeng Byeon, Chaejin Lee, Ji Heui Kim, Sang Woo Song, Chang-Ki Hong, Jeong Hoon Kim, Yong Hwy Kim, Doo-Sik Kong, Young-Hoon Kim
{"title":"Comparison of the Lumbar Drain and the Hydroxyapatite Methods for Cerebrospinal Fluid Leakage after Endoscopic Skull Base Surgery.","authors":"Minho Yang, Gung Ju Kim, Juhee Jeon, Yukyeng Byeon, Chaejin Lee, Ji Heui Kim, Sang Woo Song, Chang-Ki Hong, Jeong Hoon Kim, Yong Hwy Kim, Doo-Sik Kong, Young-Hoon Kim","doi":"10.3340/jkns.2025.0027","DOIUrl":"https://doi.org/10.3340/jkns.2025.0027","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to compare the clinical outcomes and risk of two skull base reconstruction methods after expanded endoscopic skull base approach (EEA), viz. postoperative cerebrospinal fluid (CSF) lumbar drainage (L-method) and injectable hydroxyapatite cement without lumbar drainage (H-method).</p><p><strong>Methods: </strong>We enrolled 211 consecutive patients with grade 2 or 3 intraoperative CSF leakage during EEA. The most common preoperative diagnoses were pituitary adenoma (n=62, 29%), meningioma (n=50, 24%), and craniopharyngioma (n=28, 13%). Vascularized nasoseptal flaps were used in most cases (98%). We used the L-method and H-method in 83 (39%) and 103 patients (49%), respectively.</p><p><strong>Results: </strong>The overall reconstruction-related complication and postoperative CSF leakage rates were 8% (18/211) and 6% (12/211), respectively. The complications included postoperative CSF leakage (n=12), infection (n=4), postoperative compression (n=2), and brain herniation (n=1). The postoperative complication and CSF leakage rates did not differ significantly between the L-method (12% and 10%, respectively) and H-method (8% and 4%, respectively) (p=0.326 and 0.112, respectively). Postoperative hospital stay was significantly shorter with the H-method (6.9 days) compared to the L-method (10.0 days) (p<0.001). However, the postoperative infection rate of the H-method (n=4) was higher than that of the L-method (n=0; p=0.070).</p><p><strong>Conclusion: </strong>Skull base reconstruction using hydroxyapatite effectively prevented postoperative CSF leakage and ensured patient comfort and shorter hospitalization without postoperative lumbar drainage, although postoperative infection requires consideration.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143970394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Editors' Pick in May 2025.","authors":"Hee-Jin Yang","doi":"10.3340/jkns.2025.0080","DOIUrl":"https://doi.org/10.3340/jkns.2025.0080","url":null,"abstract":"","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143996453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hyunbong Lee, Jongjoo Lee, Myung Sub Kim, Jiwook Ryu, Youngbo Shim, Yeongu Chung
{"title":"Effectiveness of Continuous Intra-Arterial Nimodipine Infusion for the Treatment of Refractory Vasospasm after Aneurysmal Subarachnoid Hemorrhage.","authors":"Hyunbong Lee, Jongjoo Lee, Myung Sub Kim, Jiwook Ryu, Youngbo Shim, Yeongu Chung","doi":"10.3340/jkns.2025.0004","DOIUrl":"https://doi.org/10.3340/jkns.2025.0004","url":null,"abstract":"<p><strong>Objective: </strong>Delayed cerebral ischemia (DCI) is a severe complication following aneurysmal subarachnoid hemorrhage (aSAH), potentially leading to functional impairments. Cerebral vasospasm (CVS) is one of the primary mechanisms of DCI. In cases of medically refractory CVS, intra-arterial (IA) nimodipine is a rescue treatment, but its effectiveness can be insufficient. We hypothesized that continuous IA nimodipine infusion (CIAN) could serve as a salvage treatment, and we evaluated its effectiveness and safety.</p><p><strong>Methods: </strong>We evaluated 274 patients with aSAH admitted between October 2017 and February 2024, identifying those who received IA nimodipine and those who also received CIAN. The modified Rankin Scale (mRS) score at discharge was assessed in the CIAN group, and patient and disease characteristics, length of stay, and discharge mRS scores were compared between the conventional IA nimodipine and the CIAN groups.</p><p><strong>Results: </strong>Of the 274 patients, 15 received IA nimodipine, and five of those underwent CIAN. More females were observed in the medically refractory CVS group compared with the non-refractory group (87% [13/15] vs. 66% [171/259]), but there was no sex difference between the CIAN and conventional IA nimodipine groups. CIAN was initiated at a mean of 9 days after the onset of aSAH and continued for 21-81 hours. Two complications were noted, including severe brain edema and suspected heparin-induced thrombocytopenia. However, radiological assessments showed no new lesions. The CIAN group exhibited a longer duration of abnormal findings on transcranial Doppler compared to the conventional IA group (16.0±10.1 vs. 9.4±7.9 days), as well as longer NCU (17.4±10.1 vs. 14.1±7.0 days) and hospital stays (46.6±28.7 vs. 29.5±14.1 days). Nonetheless, more achieved a favorable outcome (mRS≤2) in the CIAN group (80% [4/5] vs. 70% [7/10]).</p><p><strong>Conclusion: </strong>CIAN is a viable salvage treatment for refractory CVS, providing a prolonged vasodilatory effect compared to conventional IA nimodipine, with favorable outcomes.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kawngwoo Park, Sang-Don Kim, Seung-Won Choi, Dae-Hyun Kim, Eun Kyung Park, In-Suk Bae, Jin-Uk Baek, Kun Soo Lee, Sun-Ho Lee, Soo Bin Im, Youngjin Jung, Sung-Pil Joo, Man Kyu Choi, Bum-Joon Kim, Hyun Ho Choi, Junseok W Hur
{"title":"Competency-Based Neurosurgical Residency Training Program in Korea.","authors":"Kawngwoo Park, Sang-Don Kim, Seung-Won Choi, Dae-Hyun Kim, Eun Kyung Park, In-Suk Bae, Jin-Uk Baek, Kun Soo Lee, Sun-Ho Lee, Soo Bin Im, Youngjin Jung, Sung-Pil Joo, Man Kyu Choi, Bum-Joon Kim, Hyun Ho Choi, Junseok W Hur","doi":"10.3340/jkns.2024.0056","DOIUrl":"https://doi.org/10.3340/jkns.2024.0056","url":null,"abstract":"<p><p>A proposed program for competency-based neurosurgery training was presented for adaptation to a specialized project. The core of this training program is to reflect the contents of medicine that develop in the ordinary competency course necessary during the training period of residency and to systematize the training curriculum focused on competency. For that, the Development project of the competency-based training program for neurosurgical residency was conducted under the leadership of the Training Education Committee (TEC) under the Korean Neurosurgical Society (KNS), with the support of the Ministry of Health and Welfare. In this article, we introduce an educational program for a competency-oriented training system. The educational program sets the goal of the final competency that a neurosurgeon must possess and presents a list of core competencies and activities to perform for this purpose, called entrustable professional activity (EPA). The program structure includes the following domains: seven final competencies, four core competencies, ten EPAs, and 12 neurosurgical procedures. These educational programs will be uploaded to the KNS website in the future, and we would like to encourage each training hospital to reflect on them.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143997513","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yong-Jun Lee, Woong-Beom Kim, You-Sub Kim, Sung-Pil Joo
{"title":"Comparison of Surgical and Endovascular Treatments for Partially Thrombosed Intracranial Aneurysms : Insights into Recurrence and Residual Lesions.","authors":"Yong-Jun Lee, Woong-Beom Kim, You-Sub Kim, Sung-Pil Joo","doi":"10.3340/jkns.2025.0047","DOIUrl":"https://doi.org/10.3340/jkns.2025.0047","url":null,"abstract":"<p><strong>Objective: </strong>Partially thrombosed intracranial aneurysms (PTIAs) are known to occur for both large and small aneurysms; however, standardized treatment guidelines remain undefined. This study aimed to evaluate and compare the efficacy of surgical and endovascular strategies for managing PTIAs to identify the optimal treatment approach.</p><p><strong>Methods: </strong>A retrospective analysis was performed on patients diagnosed with PTIAs who underwent surgical or endovascular treatment at our institution from January 2005 to December 2022. Patients with intraluminal thrombi confirmed via brain imaging were categorized based on the treatment modality. A total of 45 patients were included. Clinical outcomes, including preoperative and postoperative modified Rankin Scale (mRS) scores, complication rates, and recurrent or remnant aneurysm rates, were reviewed to analyze the treatment results.</p><p><strong>Results: </strong>Of the 45 enrolled patients, 31 patients (68.9%) underwent surgical treatment, and 14 patients (31.1%) received endovascular treatment. Surgical approaches included direct clipping, trapping with bypass, and aneurysm wrapping. Multivariate analysis revealed a statistically significant association between the treatment modality and recurrent or remnant aneurysms (P < 0.001). However, no significant differences were identified between the two treatment groups in terms of complication rates or functional outcomes.</p><p><strong>Conclusion: </strong>In comparison to endovascular treatment, surgical management of PTIAs demonstrated superior efficacy in minimizing recurrent and remnant aneurysms. Considering the comparable rates of postoperative complications and functional outcomes, surgical treatment may be the preferred treatment strategy, particularly for younger patients with longer follow-up periods or for cases requiring decompression.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
SungSoo Bae, Seung-Jae Hyun, Jae-Koo Lee, Dae-Jean Jo, Ki-Jeong Kim
{"title":"Surgical Correction for Adolescent Idiopathic Scoliosis : A Case Series of 139 Consecutive Patients Treated in Neurosurgical Department.","authors":"SungSoo Bae, Seung-Jae Hyun, Jae-Koo Lee, Dae-Jean Jo, Ki-Jeong Kim","doi":"10.3340/jkns.2024.0224","DOIUrl":"https://doi.org/10.3340/jkns.2024.0224","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to evaluate the clinical and radiographic results of patients with adolescent idiopathic scoliosis (AIS) operated by a single neurosurgeon.</p><p><strong>Methods: </strong>A total of 139 consecutive patients with AIS who underwent corrective surgery by a single neurosurgeon were investigated between January 2011 and January 2024. Segmental instrumentation with pedicle screws was utilized for deformity correction. Demographic information, structural type of curve using Lenke classification, degree of correction achieved, total numbers of fusion levels, complications, and clinical/radiographic results were evaluated.</p><p><strong>Results: </strong>Most patients were adolescent girls (105 vs. 34) with an average age of 15.9 vs. 16.0, respectively. The average duration of follow-up was 4.5 years (0.1-13.0 years). Nine (6.6%) patients had levoscoliosis, and the remaining 130 had dextroscoliosis. Chiari malformation with syringomyelia was incidentally diagnosed in four (2.8%) patients. Of the patients having Chiari malformation, two (50%) had levoscoliosis. The mean number of fused levels was 11.2. The mean value of the Cobb angle at the major structural curvature was 63.4° (43°-125°). The major structural curve's average percentage of correction was 78.8%. The final postoperative SRS-22 average score significantly improved to 4.3±0.4 compared with the preoperative score of 3.8±0.5 (P < 0.001). After the surgery, there were no observed cases of neurological deficits or need for screw repositioning. Revision fusion extension surgery was performed on one patient to treat postoperative shoulder imbalance.</p><p><strong>Conclusion: </strong>Patients with AIS treated by a neurosurgeon had acceptable clinical and radiographic results. However, AIS surgery necessitates a fundamental comprehension of pediatric spinal deformities, which is crucial. Furthermore, spine surgeons should be careful with patients who have levoscoliosis.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143663658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neovascularization in Outer Membrane of Chronic Subdural Hematoma : A Rationale for Middle Meningeal Artery Embolization.","authors":"Hadeel M Mansour, Bipin Chaurasia","doi":"10.3340/jkns.2024.0101","DOIUrl":"10.3340/jkns.2024.0101","url":null,"abstract":"","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":"241-242"},"PeriodicalIF":1.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11924629/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142055813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Comparative Analysis of Transcription Factors TWIST2, GATA3, and HES5 in Glioblastoma Multiforme : Evaluating Biomarker Potential and Therapeutic Targets Using in Silico Methods.","authors":"Suhmi Chung, Choonghyo Kim","doi":"10.3340/jkns.2024.0149","DOIUrl":"10.3340/jkns.2024.0149","url":null,"abstract":"<p><strong>Objective: </strong>Glioblastoma multiforme (GBM) is characterized by substantial heterogeneity and limited therapeutic options. As molecular approaches to central nervous system tumors have gained prominence, this study examined the roles of three genes, TWIST2, GATA3, and HES5, known to be involved in oncogenesis, developmental processes, and maintenance of cancer stem cell properties, which have not yet been extensively studied in GBM. This study is the first to present gene expression data for TWIST2, GATA3, and HES5 specifically within the context of GBM patient survival.</p><p><strong>Methods: </strong>Gene expression data for TWIST2, GATA3, and HES5 were collected from GBM and normal brain tissues using datasets from The Cancer Genome Atlas via the Genomic Data Commons portal and the Genotype-Tissue Expression database. These data were rigorously analyzed using in silico methods.</p><p><strong>Results: </strong>All three genes were significantly more expressed in GBM tissues than in normal tissues. TWIST2 and GATA3 were linked to lower survival rates in GBM patients. Interestingly, higher HES5 levels were associated with better survival rates, suggesting a complex role that needs more investigation.</p><p><strong>Conclusion: </strong>This study shows that TWIST2, GATA3, and HES5 could help predict outcomes in GBM patients. Our multigene model offers a better understanding of GBM and points to new treatment options, bringing hope for improved therapies and patient outcomes. This research advances our knowledge of GBM and highlights the potential of molecular diagnostics in oncology.</p>","PeriodicalId":16283,"journal":{"name":"Journal of Korean Neurosurgical Society","volume":" ","pages":"202-212"},"PeriodicalIF":1.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11924635/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142502179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}