The Korean Society for Neuro-Oncology (KSNO) Guideline for the Management of Brain Tumor Patients During the Crisis Period: A Consensus Recommendation Using the Delphi Method (Version 2023.1)

Background@#During the coronavirus disease 2019 (COVID-19) pandemic, the need for appropriate treatment guidelines for patients with brain tumors was indispensable due to the lack and limitations of medical resources. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future. @*Methods@#The KSNO Guideline Working Group was composed of 22 multidisciplinary experts on neuro-oncology in Korea. In order to reach consensus among the experts, the Delphi method was used to build up the final recommendations. @*Results@#All participating experts completed the series of surveys, and the results of final survey were used to draft the current consensus recommendations. Priority levels of surgery and radiotherapy during crises were proposed using appropriate time window-based criteria for management outcome. The highest priority for surgery is assigned to patients who are life-threatening or have a risk of significant impact on a patient’s prognosis unless immediate intervention is given within 24–48 hours. As for the radiotherapy, patients who are at risk of compromising their overall survival or neurological status within 4–6 weeks are assigned to the highest priority. Curative-intent chemotherapy has the highest priority, followed by neoadjuvant/adjuvant and palliative chemotherapy during a crisis period. Telemedicine should be actively considered as a management tool for brain tumor patients during the mass infection crises such as the COVID-19 pandemic. @*Conclusion@#It is crucial that adequate medical care for patients with brain tumors is maintained and provided, even during times of crisis. This guideline will serve as a valuable resource, assisting in the delivery of treatment to brain tumor patients in the event of any future crisis.

The Korean Society for Neuro-Oncology (KSNO) Guideline for the Management of Brain Tumor Patients During the Crisis Period: A Consensus Survey About Specific Clinical Scenarios (Version 2023.1)

Background@#During the coronavirus disease 2019 (COVID-19) pandemic, there was a shortage of medical resources and the need for proper treatment guidelines for brain tumor patients became more pressing. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future. As part II of the guideline, this consensus survey is to suggest management options in specific clinical scenarios during the crisis period. @*Methods@#The KSNO Guideline Working Group consisted of 22 multidisciplinary experts on neuro-oncology in Korea. In order to confirm a consensus reached by the experts, opinions on 5 specific clinical scenarios about the management of brain tumor patients during the crisis period were devised and asked. To build-up the consensus process, Delphi method was employed. @*Results@#The summary of the final consensus from each scenario are as follows. For patients with newly diagnosed astrocytoma with isocitrate dehydrogenase (IDH)-mutant and oligodendroglioma with IDH-mutant/1p19q codeleted, observation was preferred for patients with low-risk, World Health Organization (WHO) grade 2, and Karnofsky Performance Scale (KPS) ≥60, while adjuvant radiotherapy alone was preferred for patients with high-risk, WHO grade 2, and KPS ≥60. For newly diagnosed patients with glioblastoma, the most preferred adjuvant treatment strategy after surgery was radiotherapy plus temozolomide except for patients aged ≥70 years with KPS of 60 and unmethylated MGMT promoters. In patients with symptomatic brain metastasis, the preferred treatment differed according to the number of brain metastasis and performance status. For patients with newly diagnosed atypical meningioma, adjuvant radiation was deferred in patients with older age, poor performance status, complete resection, or low mitotic count. @*Conclusion@#It is imperative that proper medical care for brain tumor patients be sustained and provided, even during the crisis period. The findings of this consensus survey will be a useful reference in determining appropriate treatment options for brain tumor patients in the specific clinical scenarios covered by the survey during the future crisis.

Immune Checkpoint Inhibitors for Non-Small-Cell Lung Cancer with Brain Metastasis : The Role of Gamma Knife Radiosurgery

: Immune checkpoint inhibitors (ICIs) are approved for treating non-small-cell lung cancer (NSCLC); however, the safety and efficacy of combined ICI and Gamma Knife radiosurgery (GKS) treatment remain undefined. In this study, we retrospectively analyzed patients treated with ICIs with or without GKS at our institute to manage patients with brain metastases from NSCLC. Methods : We retrospectively reviewed medical records of patients with brain metastases from NSCLC treated with ICIs between January 2015 and December 2017. Of 134 patients, 77 were assessable for brain responses and categorized into three groups as follows : group A, ICI alone (n=26); group B, ICI with concurrent GKS within 14 days (n=24); and group C, ICI with non-concurrent GKS (n=27). Results : The median follow-up duration after brain metastasis diagnosis was 19.1 months (range, 1–77). At the last follow-up, 53 patients (68.8%) died, 20 were alive, and four were lost to follow-up. The estimated median overall survival (OS) of all patients from the date of brain metastasis diagnosis was 20.0 months (95% confidence interval, 12.5–27.7) (10.0, 22.5, and 42.1 months in groups A, B, and C, respectively). The OS was shorter in group A than in group C (p=0.001). The intracranial disease progression-free survival (p=0.569), local progression-free survival (p=0.457), and complication rates did not significantly differ among the groups. Twelve patients showed leptomeningeal seeding (LMS) during follow-up. The 1-year LMS-free rate in treated with ICI alone group (69.1%) was significantly lower than that in treated with GKS before ICI treatment or within 14 days group (93.2%) (p=0.004). Conclusion : GKS with ICI showed no favorable OS outcome in treating brain metastasis from NSCLC. However, GKS with ICI did not increase the risk of complications. Furthermore, compared with ICI alone, GKS with ICI may be associated with a reduced incidence of LMS. Further understanding of the mechanism, which remains unknown, may help improve the quality of life of patients with brain metastasis.

Successful Pallidal Stimulation in a Patient with KMT2B-Related Dystonia

Although the KMT2B gene was identified as a causative gene for early-onset generalized dystonia, the efficacy of deep brain stimulation (DBS) in KMT2B-related dystonia has not been clearly elucidated. Here, we describe a 28-year-old woman who developed generalized dystonia with developmental delay, microcephaly, short stature, and cognitive decline. She was diagnosed with KMT2B- related dystonia using whole-exome sequencing with a heterozygous frameshift insertion of c.515dupC (p.T172fs) in the KMT2B gene. Oral medications and botulinum toxin injection were not effective. The dystonia markedly improved with bilateral pallidal DBS (the Burke-Fahn-Marsden Dystonia Rating Scale score was reduced from 30 to 5 on the dystonia movement scale and from 11 to 1 on the disability scale), and she could walk independently. From this case, we suggest that bilateral globus pallidus internus DBS can be an effective treatment option for patients with KMT2B-related generalized dystonia.

The Effect of Globus Pallidus Interna Deep Brain Stimulation on a Dystonia Patient with the GNAL Mutation Compared to Patients with DYT1 and DYT6

OBJECTIVE: The aim of this study was to investigate the efficacy of globus pallidus interna deep brain stimulation (GPi-DBS) for treating dystonia due to the GNAL mutation. METHODS: We provide the first report of a dystonia patient with a genetically confirmed GNAL mutation in the Korean population and reviewed the literature on patients with the GNAL mutation who underwent GPi-DBS. We compared the effectiveness of DBS in patients with the GNAL mutation compared to that in patients with DYT1 and DYT6 in a previous study. RESULTS: Patients with the GNAL mutation and those with DYT1 had higher early responder rates (GNAL, 5/5, 100%; DYT1, 7/7, 100%) than did patients with DYT6 (p = 0.047). The responder rates at late follow-up did not differ statistically among the three groups (p = 0.278). The decrease in the dystonia motor scale score in the GNAL group was 46.9% at early follow-up and 63.4% at late follow-up. CONCLUSION: GPi-DBS would be an effective treatment option for dystonia patients with the GNAL mutation who are resistant to medication or botulinum toxin treatment.

Gamma Knife Radiosurgery for Cancer Metastasized to the Ocular Choroid

OBJECTIVE: Choroidal metastases (CMs) are the most common intraocular tumor. Management is mainly radiation therapy with goals of pain control and visual improvement. However, many radiation-related complications are reported. Since gamma knife radiosurgery (GKS) for CM was first reported in 1995, few cases have been reported. We report 7 cases of CMs treated with GKS.METHODS: From April 2011 to November 2014, 7 patients with CM underwent GKS. Their median age at treatment was 64 years (range, 51–71 years). Four males and three females were treated. Lung cancer was the most common primary pathology, followed by renal cell carcinoma and stomach cancer. Four patients had multiple cerebral lesions and were treated simultaneously for choroidal lesions. The median marginal dose of 20 Gy (range, 15–25 Gy) was administered at the 50% isodose line.RESULTS: Median follow-up period after GKS was 8 months (range, 2–38.3 months). Four patients expired due to underlying malignancy progression. Except for two patients who were not followed with magnetic resonance image after GKS, all patients showed size reduction in the treated lesions, but a new choroidal lesion appeared in one patient and one recurred. Six of seven patients reported subjectively improved visual symptoms. Visual acuity improved in 2 patients, and 2 were stable upon objective examination. One patient showed no improvement in visual acuity, but ocular pain was relieved; another patient showed improved vision and tumor remission, but visual deterioration recurred.CONCLUSION: GKS was shown to be safe and effective and should be considered for CM treatment.

Gamma Knife Radiosurgery for Cancer Metastasized to the Ocular Choroid

OBJECTIVE: Choroidal metastases (CMs) are the most common intraocular tumor. Management is mainly radiation therapy with goals of pain control and visual improvement. However, many radiation-related complications are reported. Since gamma knife radiosurgery (GKS) for CM was first reported in 1995, few cases have been reported. We report 7 cases of CMs treated with GKS. METHODS: From April 2011 to November 2014, 7 patients with CM underwent GKS. Their median age at treatment was 64 years (range, 51–71 years). Four males and three females were treated. Lung cancer was the most common primary pathology, followed by renal cell carcinoma and stomach cancer. Four patients had multiple cerebral lesions and were treated simultaneously for choroidal lesions. The median marginal dose of 20 Gy (range, 15–25 Gy) was administered at the 50% isodose line. RESULTS: Median follow-up period after GKS was 8 months (range, 2–38.3 months). Four patients expired due to underlying malignancy progression. Except for two patients who were not followed with magnetic resonance image after GKS, all patients showed size reduction in the treated lesions, but a new choroidal lesion appeared in one patient and one recurred. Six of seven patients reported subjectively improved visual symptoms. Visual acuity improved in 2 patients, and 2 were stable upon objective examination. One patient showed no improvement in visual acuity, but ocular pain was relieved; another patient showed improved vision and tumor remission, but visual deterioration recurred. CONCLUSION: GKS was shown to be safe and effective and should be considered for CM treatment.

Outcome of Gamma Knife Thalamotomy in Patients with an Intractable Tremor

OBJECTIVE: Tremor is a common movement disorder that interferes with daily living. Since the medication for tremor has some limitations, surgical intervention is needed in many patients. In certain patients who cannot undergo aggressive surgical intervention, Gamma Knife thalamotomy (GKT) is a safe and effective alternative. METHODS: From June 2012 to August 2013, 7 patients with an intractable tremor underwent GKT. Four of these 7 patients had medical comorbidities, and 3 patients refused to undergo traditional surgery. Each patient was evaluated with the modified Fahn-Tolosa-Marin tremor rating scale (TRS) along with analysis of handwriting samples. All of the patients underwent GKT with a maximal dose of 130 Gy to the left ventralis intermedius (VIM) nucleus of the thalamus. Follow-up brain MRI was performed after 3 to 8 months of GKT, and evaluation with the TRS was also performed. RESULTS: Six patients showed objective improvement in the TRS score. Excluding one patient who demonstrated tremor progression, there was 28.9% improvement in the TRS score. However, five patients showed subjective improvement in their symptoms. On comparing the TRS scores between follow-up periods of more and less than 4 months, the follow-up TRS score at more than 4 months of GKT was significantly improved compared to that at less than 4 months of GKT. Follow-up MRI showed radiosurgical changes in 5 patients. CONCLUSION: GKT with a maximal dose of 130 Gy to the VIM is a safe procedure that can replace other surgical procedures.

Microsurgical Treatment and Outcome of Pediatric Supratentorial Cerebral Cavernous Malformation

OBJECTIVE: The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system. METHODS: Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed. RESULTS: The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness. CONCLUSION: With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary.

Mid-term Clinical Outcomes of Stand-alone Posterior Interbody Fusion with Rectangular Cages: A 4-year-minimum Follow-up

OBJECTIVE: We sought to determine minimum 4 years of clinical outcomes including fusion rate, revision rate and complications of patients who underwent placement of rectangular stand-alone cages. METHODS: Thirty-three cases of degenerative spine that had been followed for at least 4-years were reviewed retrospectively. Cages were inserted at L4-L5 level or L5-S1 in 27 or in 6 cases respectively. Visual analogue scale (VAS), Odom's criteria, fusion rate, intervertebral disc height and lumbar lordosis were determined pre- and post-operatively on standing x-rays. Amount of intra- and postoperative blood loss, total volume transfused, duration of surgery and perioperative complications were also evaluated. RESULTS: The mean VAS score of back pain and sciatica were improved from 8.0 and 7.0 points to 3.4 and 2.4 during 1 years follow-up visit and the scores was raised gradually. Also, during the follow-up, 94% of patients showed excellent or good outcomes by the Odom's criteria. Intervertebral disc height was increased from 8.2+/-1.4mm to 9.2+/-1.9mm at the first year of follow-up, however, found to be decreased and stabilized to 8.3+/-1.8mm after 2 years. The fusion rate was approximately 91% after 4 year postoperative. The segmental angle of lordosis was increased significantly by two years but it was not maintained after four years. A statistically insignificant change in total lumbar lordosis was also observed. Three patients (9%) had experienced perioperative complications. CONCLUSION: The use of rectangular stand-alone cages for posterior lumbar interbody fusion (PLIF) resulted in a various degree of subsidence and demonstrate very low complication rate, high functional stability and improved clinical outcomes in patients with degenerative lumbar disc disease.

Bromocriptine Therapy for the Treatment of Invasive Prolactinoma: The Single Institute Experience

OBJECTIVE: The objective of this study was to describe and characterize the clinical course of treatment for invasive prolactinoma patients using bromocriptine. METHODS: The study group included 23 patients who were treated with bromocriptine for their invasive prolactinomas. Clinical histories, serum prolactin level and pituitary hormone assessments, tumor diameter and signal intensity on sella magnetic resonance imaging (MRI), visual field exams and the dosage of medications were reviewed for each patient. RESULTS: During 30 months (median, range 6-99) of follow-up period, 19 patients treated with bromocriptine alone achieved biochemical remission. Four patients changed the medication to cabergoline due to the adverse effects or observed resistance of bromocriptine treatment. All of five patients who had visual symptoms improved after the course of medication. Four surgically treated patients were not able to discontinue medication because they could not maintain biochemical remission state without medication. Multivariate analysis showed that decreased enhancement on the initial followed MRI after medication and longer follow-up periods were associated with higher radiologic response. CONCLUSION: We reassure that the dopamine agonist is safe and effective for the treatment of invasive pituitary adenomas. Meanwhile, surgery has a limited role on biochemical remission. Decreased enhancement on the initial follow-up MRI after medication may reflect the treatment response. Further study is required to validate the role of MRI or other factors on the actual prognosis.

Upward Migration of a Peritoneal Catheter Following Ventriculoperitoneal Shunt

We present an unusual case of peritoneal catheter migration following a ventriculoperitoneal shunt operation. A 7-month-old infant, who had suffered from intraventricular hemorrhage at birth, was shunted for progressive hydrocephalus. The peritoneal catheter, connected to an 'ultra small, low pressure valve system' (Strata(R); PS Medical,Gola, CA, USA) at the subgaleal space, was placed into the peritoneal cavity about 30 cm in length. The patient returned to our hospital due to scalp swelling 21 days after the surgery. Simple X-ray images revealed total upward migration and coiling of the peritoneal catheter around the valve. Possible mechanisms leading to proximal upward migration of a peritoneal catheter are discussed.

Change of Selection to Antihypertensive Drugs in Hypertensive Patients with Diabetes Mellitus: In Pohang . Gyeongju Primary Care Research Network / 가정의학회지

BACKGROUND: Angiotensin converting enzyme inhibitors (ACEIs) or Angiotensin II type 1 receptor blockers (ARBs) are compelling indication drugs for hypertensive patients with diabetes mellitus. But prescription rate in 2005 year study of Pohang . Gyeongju area was only 30.8%. Therefore, a study on the change of prescription rate in the same area after 3 years was done. METHODS: During three months from January 2008, 152 hypertensive patients with diabetes mellitus on their prescribed antihypertensive medications by 9 family physicians in visiting order were analyzed. After the analysis, the infl uencing factors for such prescriptions were ascertained by directly visiting each physicians who prescribed them. RESULTS: A regimen of 16 antihypertensive agents were prescribed by these family physicians. Prescription count of ACEIs or ARBs was 101 cases (66.4%). ACEIs single therapy was 19 cases (12.5%), ACEIs combination therapy was 7 cases (4.6%), ARBs single therapy was 31 cases (20.4%) and ARBs combination therapy was 44 cases (28.9%). The ACEIs or ARBs which were selected by physicians that followed "compelling indication" was 5 (55.6%), "excellent reduce pressure effect" was 3 (33.3%) and "public relations of new medicine" was 1 (11.1%). CONCLUSION: In prescribing antihypertensive agents for patients with diabetes mellitus, selection of ACEIs or ARBs was increased from 30.8% to 66.4%. Education of recommended standard by participating in such study and developing of excellent new medicines may increase such change.

Selection of Antihypertensive Drugs in Hypertensive Patients with Diabetes Mellitus: In Pohang-Gyeongju Primary Care Research Network

BACKGROUND: Angiotensin converting enzyme inhibitor or Angiotensin II type 1 receptor blocker is usually recommended for hypertensive patients with diabetes mellitus. No reports have been made on antihypertensive agents prescribed for these patients in private practice. METHODS: During a one month period of December 2004, 169 hypertensive patients with diabetes mellitus on their prescribed antihypertensive agents by ten family physicians were analyzed. The influencing factors for such prescriptions were analyzed by directly visiting each physicians who prescribed them. RESULTS: A total of 21 antihypertensive agents were prescribed by these family physicians. Single calcium channel blocker therapy was the most common with 55 cases (32.5%), followed by 20 cases (11.8%) of single angiotensin converting enzyme inhibitor therapy and 16 cases of single therapy of Angiotensin II type 1 receptor blocker. The low frequency of prescribing the Angiotensin II type 1 receptor blocker was due to insufficient effect (7), lack of information (5), resistance from the patients from changing the medications (4) and expensive costs (4). CONCLUSION: In prescribing antihypertensive agents for patients with diabetes mellitus, angiotensin converting enzyme inhibitor or Angiotensin II type 1 receptor blocker were chosen less. In order to decrease the incidence of complications in these patients, such agents should be prescribed more.

A Case of Pulmonary Septic Emboli after Removal of BCGoma / 소아알레르기및호흡기학회지

Septic pulmonary emboli occur when thrombi contain organisms such as bacteria, fungi, or parasites. The septic emboli reach the lung from a variety of sources, including infected venous catheter or pacemaker wires, right-sided bacterial endocarditis, sepsis, septic thrombophlebitis, osteomyelitis, peritonsillar and subcutaneous abscess. Early clinical detection, along with prompt administration of broad-spectrum antibiotics, is an important factor in the prognosis of patients, but unfortunately initial clinical diagnosis is often difficult because of nonspecific findings in clinical, laboratory data. But recently, the characteristic chest computed tormography (CT) and chest radiographic findings of septic emboli have been helpful in non-invasive diagnostic method of early detection. Especially, chest CT is an important method for confirming the presence of septic pulmonary emboli. We experienced a case of septic pulmonary embolism in a 5-month-old infant with fever and intermittent cough after removal of BCGoma. The diagnosis was made on the basis of a chest x-ray and a chest CT. The CT revealed the presence of multiple subpleural nodules, cavitation of nodules and feeding vessel signs. Methicillin resistant staphylococcus aureus was cultured in the wound swab culture on left axillary area. After administration of broad spectrum antibiotics, clinical and radiologic improvements were achieved.

Clinical Observation of Congenital Aganglionic Megacolon in Infancy and Childhood

This clinical observation was carried out 24 cases of the congenital aganglionic megacolon in infancy and childhood admitted to Chonnam University Hospital during 10 years from 1965 to June 1976. The following results were obtained: 1) The frequency was 1 to 500 admission patients. 2) Male to female ratio was 5 to 1 3) The familial ccurrence was not observed in this study and all infants were normal fullterm delivery. 4) The associated congenitalanomalies were mental retardation 2, inguinal hernia 1 and mongolism 1. 5) In majority of the cases, the symptoms occured oon after birth. 6) The clinical symptoms were abdominal distension(87.5%), vomiting(62.5%), constipation(62.5%), malnutrition(41.6%) and diarrhea(41.6%) in order of frequency. 7) The meconium was evacuated after digitalexamination or enema in 61.1%, and it passed spontaneously in remainder. 8) Positive findings of barium enema was noted as 26.7% in the group under the age of 2 months and 100% in the group over the age of 2 months. 9) The mortalityrate was 28.5% in the cases who had Swenson's pull-h-rough operation at the time of admission, and 20% in the case who had Swenson's pperation 6 or 12 months later after colostomy.