Efficacy of the Ketogenic Diet for Pediatric Epilepsy According to the Presence of Detectable Somatic mTOR Pathway Mutations in the Brain

Background@#and Purpose A multifactorial antiepileptic mechanism underlies the ketogenic diet (KD), and one of the proposed mechanisms of action is that the KD inhibits the mammalian target of rapamycin (mTOR) pathway. To test this clinically, this study aimed to determine the efficacy of the KD in patients with pathologically confirmed focal cortical dysplasia (FCD) due to genetically identifiable mTOR pathway dysregulation. @*Methods@#A cohort of patients with pathologically confirmed FCD after epilepsy surgery and who were screened for the presence of germline and somatic mutations related to the mTOR pathway in peripheral blood and resected brain tissue was constructed prospectively. A retrospective review of the efficacy of the prior KD in these patients was performed. @*Results@#Twenty-five patients with pathologically confirmed FCD and who were screened for the presence of detectable somatic mTOR pathway mutations had received a sufficient KD. Twelve of these patients (48.0%) had germline or somatic detectable mTOR pathway mutations. A response was defined as a ≥50% reduction in seizure frequency. The efficacy of the KD after 3 months of dietary therapy was superior in patients with detectable mTOR pathway mutations than in patients without detectable mTOR pathway mutations, although the difference was not statistically significant (responder rates of 58.3% vs. 38.5%, p=0.434). @*Conclusions@#A greater proportion of patients with mTOR pathway responded to the KD, but there was no statistically significant difference in efficacy of the KD between patients with and without detectable mTOR pathway mutations. Further study is warranted due to the smallness of the sample and the limited number of mTOR pathway genes tested in this study.

Cosmetic outcome after electrocautery versus non-electrocautery dissection of the temporalis muscle for pterional craniotomy

Objective@#Despite the usefulness of pterional craniotomy (PC), its cosmetic outcome is questionable. Electrocautery (EC) causes injuries to adjacent structures, and it could be a factor that affects the cosmetic outcome. Evaluation of cosmetic outcome is difficult because it is often determined by patient’s subjective criteria. The objective of this study is to compare the cosmetic outcome after EC versus non-electrocautery (NEC) dissection of the temporalis muscle for PC by analyzing long-term follow-up data determined from both physician and patient’s aspects. @*Methods@#Patients at follow-ups between January 2014 and April 2021 after PCs were enrolled. The keyhole (KH) site, the inferior margin of the temporal line of the frontal bone (ITL), the mid-temporal (mid-T) area, and the posterior incision line (PIL) were inspected by a physician to check the presence of depressions. Patient’s cosmetic satisfaction was categorized into satisfactory, intermediate, or unsatisfactory by a survey. The presence of osteolysis was checked from the radiological images. Patients were classified into two groups; one with EC dissection and another with NEC retrograde dissection using a double-ended dissector. @*Results@#The incidences of depression at the mid-T area and osteolysis were higher in the EC group (p=0.001, p＜0.001). The percentage of satisfactory cosmetic outcome was lower in the EC group (p=0.002). The presences of depression at the mid-T area and osteolysis were related with lower rate of satisfactory outcomes (p＜0.001, p＜0.001). @*Conclusions@#NEC dissection causes less destruction to adjacent structures and brings better cosmetic outcome after PC.

Ethanol Extract of Chaenomeles sinensis Inhibits the Development of Benign Prostatic Hyperplasia by Exhibiting Anti-oxidant and Anti-inflammatory Effects

Chaenomeles sinensis is known to inhibit the development and progression of many age-related diseases, but the underlying molecular mechanisms are largely unclear. In the present study, we observed that the ethanol extract of Chaenomeles sinensis scavenged 2,2’-diphenylpicrylhydrazyl and 2,2’-azinobis diammonium radicals in vitro. The ethanol extract of Chaenomeles sinensisactivated antioxidant response element-luciferase activity and induced expression of NRF2 target genes in HaCaT cells. The ethanol extract of Chaenomeles sinensis also suppressed LPS-induced expression of COX-2 and iNOS proteins, and mRNA expression of TNF-α and IL-2 in RAW264.7 cells. Finally, the ethanol extract of Chaenomeles sinensis significantly suppressed testosterone propionate-induced benign prostatic hyperplasia in mice. Together, our study provides the evidence that the ethanol extract of Chaenomeles sinensis inhibits the development of benign prostatic hyperplasia by exhibiting anti-oxidant and anti-inflammatory effects.

Osmotic Demyelination Syndrome Associated with Hypernatremia Caused by Lactulose Enema in a Patient with Chronic Alcoholism

A 44-year-old man with chronic alcoholism presented with seizure and loss of consciousness. He was diagnosed with alcoholic hepatic encephalopathy, and his neurologic symptoms recovered after lactulose enema treatment. His initial serum sodium level was 141 mEq/L. However, his mental state became confused after treatment with lactulose enema for five days, and his serum sodium level increased to 178 mEq/L. After five days of gradual correction of serum sodium level from 178 mEq/L to 140 mEq/L, the patient’s mental state recovered, but motor weakness in both limbs remained. Therefore, magnetic resonance imaging of the brain was performed. T2-weighted brain images showed bilateral symmetrical hyperintensities in the central pons, basal ganglia, thalami, hippocampi and unci, which were consistent with central pontine and extrapontine myelinolysis. We report a rare case of osmotic demyelination syndrome that occurred as a result of a rapid increase from a normal sodium level to hypernatremia caused by lactulose enema administered to treat alcoholic hepatic encephalopathy.

Pure Cortical Ependymoma With RELA-Fusion Positive in a Young Adult

A 25-year-old female presented with a generalized tonic-clonic seizure. She had no previous history of seizures. A brain magnetic resonance imaging scan revealed a solitary enhancing mass in the right fronto-parietal cortex. During surgery, the mass was noted to be pure cortical with no connection to the ventricular lining. The tumor was completely resected. After surgery, the patient had no further seizures. The biopsy result showed a supratentorial ependymoma, which was C11orf95-RELA-fusionpositive.

Glioblastoma Cellular Origin and the Firework Pattern of Cancer Genesis from the Subventricular Zone

Glioblastoma (GBM) is a disease without any definite cure. Numerous approaches have been tested in efforts to conquer this brain disease, but patients invariably experience recurrence or develop resistance to treatment. New surgical tools, carefully chosen samples, and experimental methods are enabling discoveries at single-cell resolution. The present article reviews the cell-of-origin of isocitrate dehydrogenase (IDH)-wildtype GBM, beginning with the historical background for focusing on cellular origin and introducing the cancer genesis patterned on firework. The authors also review mutations associated with the senescence process in cells of the subventricular zone (SVZ), and biological validation of somatic mutations in a mouse SVZ model. Understanding GBM would facilitate research on the origin of other cancers and may catalyze the development of new management approaches or treatments against IDH-wildtype GBM.

Primary Gliosarcoma with Extracranial Metastasis

A 69-year-old male presented with a week of worsening headache, mild dizziness and left side weakness,and the radiological work-up of his brain displayed an enhancing mass on the right frontal lobe.The tumor was totally resected. The patient was initially diagnosed with glioblastoma multiforme. Hisneurologic symptoms recovered after surgery. He underwent adjuvant radiotherapy with concurrent temozolomide.Approximately 7 months after surgery, the patient complained of epigastric pains. AbdominalCT scan showed multiple hepatic metastasis and multiple lymphadenopathy. Chest CT andTorso positron emission tomography-CT scans for additional metastasis study revealed multiple metastaticlesions in the right lung, left pleura, liver, lymph nodes, bones, and muscles. Percutaneous liverbiopsy was performed, and associated pathology was consistent with sarcomatous component. Afterliver biopsy, brain tumor pathology was reviewed, which revealed typical gliomatous and sarcomatouscomponents. The patient was therefore diagnosed with metastatic gliosarcoma. The patient was in aseptic condition with aggravated pleural effusion. The patient died 9 months after the diagnosis of primarygliosarcoma.

Usefulness of Allogenic Acellular Dermal Matrix for Prevention of Scalp Depressio / 대한신경손상학회지

Objective@#Burr hole trephination is a common treatment for chronic subdural hematoma, intracranial hematoma, and intraventricular hematoma due to its effective drainage of hematoma, minimal invasiveness and short operation time. However, cosmetic complications such as scalp depression can occur. The aim of this study was to evaluate the usefulness of an allogenic acellular dermal matrix (ADM) to prevent scalp depression at the burr hole site. @*Methods@#A retrospective analysis was performed with 75 cases in 66 patients who were treated with burr hole trephination from January 2018 to December 2019. These cases divided into 2 groups; based on the method used to cover the burr hole site: Gelfoam packing only (GPO) and ADM. The degree of the scalp depression was measured from the more recent follow-up brain computed tomography scan. @*Results@#There was a significant difference in the degree of scalp depression between GPO and ADM groups (p=0.003). No significant correlation between patient's age and the degree of scalp depression (GPO: p=0.419, ADM: p=0.790). There were no wound infection complication in either group. @*Conclusion@#ADM is a suitable material to prevent scalp depression after burr hole trephination.

A Case of Recurrent Supratentorial Extraventricular Anaplastic Ependymoma in Adult

Supratentorial extraventricular anaplastic ependymoma (SEAE) in adults is a relatively rare intracranial tumor. Because of the very low prevalence, only a few cases have been reported. According to a recent study, SEAE is associated with a poor prognosis and there is no definite consensus on optimal treatment. We report a case of an adult SEAE patient who had no recurrence until seven years after a gross total resection (GTR) followed by conventional radiotherapy. A 42-year-old male had a persistent mild headache, left facial palsy, dysarthria, and left hemiparesis. Preoperative neuroimaging revealed an anaplastic astrocytoma or supratentorial ependymoma in the right frontal lobe. A GTR was performed, followed by adjuvant radiotherapy. Histologic and immunohistochemical results revealed anaplastic ependymoma. After seven years of initial therapy, a regular follow-up MRI showed a 3-cm-sized partially cystic mass in the same area as the initial tumor. The patient underwent a craniotomy, and a GTR was performed. Histopathologic examination revealed recurrence of the SEAE. External radiotherapy was performed. The patient has been stable without any disease progression or complications for 12 months since the surgery for recurrent SEAE.

Mechanistic Target of Rapamycin Pathway in Epileptic Disorders

The mechanistic target of rapamycin (mTOR) pathway coordinates the metabolic activity of eukaryotic cells through environmental signals, including nutrients, energy, growth factors, and oxygen. In the nervous system, the mTOR pathway regulates fundamental biological processes associated with neural development and neurodegeneration. Intriguingly, genes that constitute the mTOR pathway have been found to be germline and somatic mutation from patients with various epileptic disorders. Hyperactivation of the mTOR pathway due to said mutations has garnered increasing attention as culprits of these conditions : somatic mutations, in particular, in epileptic foci have recently been identified as a major genetic cause of intractable focal epilepsy, such as focal cortical dysplasia. Meanwhile, epilepsy models with aberrant activation of the mTOR pathway have helped elucidate the role of the mTOR pathway in epileptogenesis, and evidence from epilepsy models of human mutations recapitulating the features of epileptic patients has indicated that mTOR inhibitors may be of use in treating epilepsy associated with mutations in mTOR pathway genes. Here, we review recent advances in the molecular and genetic understanding of mTOR signaling in epileptic disorders. In particular, we focus on the development of and limitations to therapies targeting the mTOR pathway to treat epileptic seizures. We also discuss future perspectives on mTOR inhibition therapies and special diagnostic methods for intractable epilepsies caused by brain somatic mutations.

Mechanistic Target of Rapamycin Pathway in Epileptic Disorders

The mechanistic target of rapamycin (mTOR) pathway coordinates the metabolic activity of eukaryotic cells through environmental signals, including nutrients, energy, growth factors, and oxygen. In the nervous system, the mTOR pathway regulates fundamental biological processes associated with neural development and neurodegeneration. Intriguingly, genes that constitute the mTOR pathway have been found to be germline and somatic mutation from patients with various epileptic disorders. Hyperactivation of the mTOR pathway due to said mutations has garnered increasing attention as culprits of these conditions : somatic mutations, in particular, in epileptic foci have recently been identified as a major genetic cause of intractable focal epilepsy, such as focal cortical dysplasia. Meanwhile, epilepsy models with aberrant activation of the mTOR pathway have helped elucidate the role of the mTOR pathway in epileptogenesis, and evidence from epilepsy models of human mutations recapitulating the features of epileptic patients has indicated that mTOR inhibitors may be of use in treating epilepsy associated with mutations in mTOR pathway genes. Here, we review recent advances in the molecular and genetic understanding of mTOR signaling in epileptic disorders. In particular, we focus on the development of and limitations to therapies targeting the mTOR pathway to treat epileptic seizures. We also discuss future perspectives on mTOR inhibition therapies and special diagnostic methods for intractable epilepsies caused by brain somatic mutations.

Longevity and Stress Resistant Property of 6-Gingerol from Zingiber officinale Roscoe in Caenorhabditis elegans

In order to discover lifespan-extending compounds made from natural resources, activity-guided fractionation of Zingiber officinale Roscoe (Zingiberaceae) ethanol extract was performed using the Caenorhabditis elegans (C. elegans) model system. The compound 6-gingerol was isolated from the most active ethyl acetate soluble fraction, and showed potent longevity-promoting activity. It also elevated the survival rate of worms against stressful environment including thermal, osmotic, and oxidative conditions. Additionally, 6-gingerol elevated the antioxidant enzyme activities of C. elegans, and showed a dose-depend reduction of intracellular reactive oxygen species (ROS) accumulation in worms. Further studies demonstrated that the increased stress tolerance of 6-gingerol-mediated worms could result from the promotion of stress resistance proteins such as heat shock protein (HSP-16.2) and superoxide dismutase (SOD-3). The lipofuscin levels in 6-gingerol treated intestinal worms were decreased in comparison to the control group. No significant 6-gingerol-related changes, including growth, food intake, reproduction, and movement were noted. These results suggest that 6-gingerol exerted longevity-promoting activities independently of these factors and could extend the human lifespan.

A Rare Case of Subarachnoid Hemorrhage caused by Ruptured Venous Varix Due to Dural Arteriovenous Fistula at the Foramen Magnum Fed Solely by the Ascending Pharyngeal Artery

Dural arteriovenous fistula (D-AVF) at the foramen magnum is an extremely rare disease entity. It produces venous hypertension, and can lead to progressive cervical myelopathy thereafter. On the other hand, the venous hypertension may lead to formation of a venous varix, and it can rarely result in an abrupt onset of subarachnoid hemorrhage (SAH) when the venous varix is ruptured. The diagnosis of D-AVF at the foramen magnum as a cause of SAH may be difficult due to its low incidence. Furthermore, when the D-AVF is fed solely by the ascending pharyngeal artery (APA), it may be missed if the external carotid angiography is not performed. The outcome could be fatal if the fistula is unrecognized. Herein, we report on a rare case of SAH caused by ruptured venous varix due to D-AVF at the foramen magnum fed solely by the APA. A review of relevant literatures is provided, and the treatment modalities and outcomes are also discussed.

Predictable Values of Decompressive Craniectomy in Patients with Acute Subdural Hematoma: Comparison between Decompressive Craniectomy after Craniotomy Group and Craniotomy Only Group / 대한신경손상학회지

OBJECTIVE: Patients with traumatic acute subdural hematoma (ASDH) often require surgical treatment. Among patients who primarily underwent craniotomy for the removal of hematoma, some consequently developed aggressive intracranial hypertension and brain edema, and required secondary decompressive craniectomy (DC). To avoid reoperation, we investigated factors which predict the requirement of DC by comparing groups of ASDH patients who did and did not require DC after craniotomy. METHODS: The 129 patients with ASDH who underwent craniotomy from September 2007 to September 2017 were reviewed. Among these patients, 19 patients who needed additional DC (group A) and 105 patients who underwent primary craniotomy only without reoperation (group B) were evaluated. A total of 17 preoperative and intraoperative factors were analyzed and compared statistically. Univariate and multivariate analyses were used to compare these factors. RESULTS: Five factors showed significant differences between the two groups. They were the length of midline shifting to maximal subdural hematoma thickness ratio (magnetization transfer [MT] ratio) greater than 1 (p 1, IVH, and TICH on preoperative brain computed tomography images, intraoperative signs of intracranial hypertension, brain edema, and bleeding tendency were identified as factors indicating that DC would be required. The necessity for preemptive DC must be carefully considered in patients with such risk factors.

Acute Kidney Injury Resulting from Amanita neoovoidea Intoxication

No abstract available.

Panax Ginseng Induces Toxic Hepatitis and Acute Kidney Injury / 전남의대학술지

No abstract available.

Panax Ginseng Induces Toxic Hepatitis and Acute Kidney Injury / 전남의대학술지

No abstract available.

Biomarkers Predicting Survival of Sepsis Patients Treated with Continuous Renal Replacement Therapy / 전남의대학술지

The present study investigated the prognostic factors predicting survival of patients with sepsis and acute kidney injury (AKI) undergoing continuous renal replacement therapy (CRRT). This retrospective observational study included 165 sepsis patients treated with CRRT. The patients were divided into two groups; the survivor group (n=73, 44.2%) vs. the nonsurvivor group (n=92, 55.8%). AKI was defined by the 2012 Kidney Disease: Improving Global Outcomes Clinical Practice Guidelines. We analyzed medical histories, clinical characteristics and laboratory findings of the enrolled patients when they started CRRT. In addition, we performed binary logistic regression and cox regression analysis. In the survivor group, urine output during the first day was significantly higher compared with the nonsurvivor group (55.7±66.3 vs. 26.6±46.4, p=0.001). Patients with urine output <30 mL/hour during the 1st day showed worse outcomes than ≥30 mL/hour in the logistic regression (hazard ratio 2.464, 95% confidence interval 1.152-5.271, p=0.020) and the cox regression analysis (hazard ratio 1.935, 95% confidence interval 1.147-3.263, p=0.013). In conclusion, urine output may predict survival of septic AKI patients undergoing CRRT. In these patients, urine output <30 mL/hour during the first day was the strongest risk factor for in-hospital mortality.

Biomarkers Predicting Survival of Sepsis Patients Treated with Continuous Renal Replacement Therapy / 전남의대학술지

The present study investigated the prognostic factors predicting survival of patients with sepsis and acute kidney injury (AKI) undergoing continuous renal replacement therapy (CRRT). This retrospective observational study included 165 sepsis patients treated with CRRT. The patients were divided into two groups; the survivor group (n=73, 44.2%) vs. the nonsurvivor group (n=92, 55.8%). AKI was defined by the 2012 Kidney Disease: Improving Global Outcomes Clinical Practice Guidelines. We analyzed medical histories, clinical characteristics and laboratory findings of the enrolled patients when they started CRRT. In addition, we performed binary logistic regression and cox regression analysis. In the survivor group, urine output during the first day was significantly higher compared with the nonsurvivor group (55.7±66.3 vs. 26.6±46.4, p=0.001). Patients with urine output <30 mL/hour during the 1st day showed worse outcomes than ≥30 mL/hour in the logistic regression (hazard ratio 2.464, 95% confidence interval 1.152-5.271, p=0.020) and the cox regression analysis (hazard ratio 1.935, 95% confidence interval 1.147-3.263, p=0.013). In conclusion, urine output may predict survival of septic AKI patients undergoing CRRT. In these patients, urine output <30 mL/hour during the first day was the strongest risk factor for in-hospital mortality.

Shunt Overdrainage Caused by Displacement of the Pressure Control Cam after Pressure Adjustment / 대한신경손상학회지

Although the Codman-Hakim programmable valve is one of most popular shunt systems used in the clinical practice for the treatment of hydrocephalus, malfunctions related with this system have been also reported which lead to underdrainage or overdrainage of the cerebrospinal fluid. While obstruction of the ventricular catheter by tissue materials or hematoma and catheter disconnection are relatively common, the malfunction of the valve itself is rare. Herein, we report on a rare case of shunt overdrainage caused by displacement of the pressure control cam after pressure adjustment. A 57-year-old female, who underwent a ventriculoperitoneal shunt eight years ago, experienced aggravating symptoms of shunt overdrainage after pressure adjustment. Displacement of the pressure control cam was revealed on the X-ray, and a shunt revision was performed. The purpose of this report is to provide a working knowledge of the valve structure and to enhance the ability to interpret the valve setting on an X-ray for diagnosis of valve malfunction.