Clinical Effect of Transverse Process Hook with K-Means Clustering-Based Stratification of Computed Tomography Hounsfield Unit at Upper Instrumented Vertebra Level in Adult Spinal Deformity Patients

Objective@#: This study aimed to investigate the efficacy of transverse process (TP) hook system at the upper instrumented vertebra (UIV) for preventing screw pullout in adult spinal deformity surgery using the pedicle Hounsfield unit (HU) stratification based on K-means clustering. @*Methods@#: We retrospectively reviewed 74 patients who underwent deformity correction surgery between 2011 and 2020 and were followed up for >12 months. Pre- and post-operative data were used to determine the incidence of screw pullout, UIV TP hook implementation, vertebral body HU, pedicle HU, and patient outcomes. Data was then statistically analyzed for assessment of efficacy and risk prediction using stratified HU at UIV level alongside the effect of the TP hook system. @*Results@#: The screw pullout rate was 36.4% (27/74). Perioperative radiographic parameters were not significantly different between the pullout and non-pullout groups. The vertebral body HU and pedicle HU were significantly lower in the pullout group. K-means clustering stratified the vertebral body HU ≥205.3, <137.2, and pedicle HU ≥243.43, <156.03. The pullout rate significantly decreases in patients receiving the hook system when the pedicle HU was from ≥156.03 to < 243.43 (p<0.05), but the difference was not statistically significant in the vertebra HU stratified groups and when pedicle HU was ≥243.43 or <156.03. The postoperative clinical outcomes improved significantly with the implementation of the hook system. @*Conclusion@#: The UIV hook provides better clinical outcomes and can be considered a preventative strategy for screw-pullout in the certain pedicle HU range.

Radiological Changes in Adjacent and Index Levels after Cervical Disc Arthroplasty

Purpose@#The purpose of this retrospective study was to evaluate radiological and clinical outcomes in patients undergoing cervical disc arthroplasty (CDA) for cervical degenerative disc disease. The results may assist in surgical decision-making and enable more effective and safer implementation of cervical arthroplasty. @*Materials and Methods@#A total of 125 patients who were treated with CDA between 2006 and 2019 were assessed. Radiological measurements and clinical outcomes included the visual analogue scale (VAS), the Neck Disability Index (NDI), and the Japanese Orthopaedic Association (JOA) myelopathy score assessment preoperatively and at ≥2 years of follow-up. @*Results@#The mean follow-up period was 38 months (range, 25–114 months). Radiographic data demonstrated mobility at both the index and adjacent levels, with no signs of hypermobility at an adjacent level. There was a non-significant loss of cervical global motion and range of motion (ROM) of the functional spinal unit at the operated level, as well as the upper and lower adjacent disc levels, compared to preoperative status. The cervical global and segmental angle significantly increased. Postoperative neck VAS, NDI, and JOA scores showed meaningful improvements after one- and two-level CDA. We experienced a 29.60% incidence of heterotrophic ossification and a 3.20% reoperation rate due to cervical instability, implant subsidence, or osteolysis. @*Conclusion@#CDA is an effective surgical technique for optimizing clinical outcomes and radiological results. In particular, the preservation of cervical ROM with an artificial prosthesis at adjacent and index levels and improvement in cervical global alignment could reduce revision rates due to adjacent segment degeneration.

Short Communication: Links between Dental Hygiene Curriculum and Dental Hygiene Task Analysis / 치위생과학회지

Background@#The problem with current dental hygienist education is that it operates as an education system based on the national examination rather than on a practical basis; thus, graduates have difficulties in practice after obtaining their license. This study aimed to propose a job-oriented curriculum by analyzing the links between the task analysis of Korean dental hygienists and dental hygiene learning goals. @*Methods@#This study performed a relationship analysis based on a second job analysis study of dental hygienists conducted by the Korea Health Personnel Licensing Examination Institute and the learning goals of the Korean Dental Hygiene Faculty Association. @*Results@#Based on the links between the task and learning goals of the dental hygienist, they were classified into six types: 1) tasks listed in the license exam and learning goal, 2) tasks not listed in the license exam but listed in learning goals, 3) tasks not listed in learning goals, 4) learning goals not related to tasks, 5) learning goals listed in a few tasks, and 6) tasks related to several learning goals. The results showed that most of them correspond to the 5th classification, followed by the 3rd and 4th categories, which are mostly basic science learning goals. Tasks without learning goals are not included in the curriculum; thus, the curriculum needs to be supplemented. The overlapping learning goals of several subjects for one job skill must be reduced in job-oriented education. @*Conclusion@#We suggest that the dental hygiene curriculum be developed based on task analysis and reflected in the national dental hygienist exam. The clinical practice performance of dental hygienists will take further leap forward through task-oriented education.

Preoperative Radiological Parameters to Predict Clinical and Radiological Outcomes after Laminoplasty

Many studies have focused on pre-operative sagittal alignment parameters which could predict poor clinical or radiological outcomes after laminoplasty. However, the influx of too many new factors causes confusion. This study reviewed sagittal alignment parameters, predictive of clinical or radiological outcomes, in the literature. Preoperative kyphotic alignment was initially proposed as a predictor of clinical outcomes. The clinical significance of the K-line and K-line variants also has been studied. Sagittal vertical axis, T1 slope (T1s), T1s-cervical lordosis (CL), anterolisthesis, local kyphosis, the longitudinal distance index, and range of motion were proposed to have relationships with clinical outcomes. The relationship between loss of cervical lordosis (LCL) and T1s has been widely studied, but controversy remains. Extension function, the ratio of CL to T1s (CL/T1s), and Sharma classification were recently proposed as LCL predictors. In predicting postoperative kyphosis, T1s cannot predict postoperative kyphosis, but a low CL/T1s ratio was associated with postoperative kyphosis.

Factors Affecting Postoperative Complications and Outcomes of Cervical Spondylotic Myelopathy with Cerebral Palsy : A Retrospective Analysis

Objective@#: Cervical surgery in patients with cervical spondylotic myelopathy (CSM) and cerebral palsy (CP) is challenging owing to the complexities of the deformity. We assessed factors affecting postoperative complications and outcomes after CSM surgery in patients with CP. @*Methods@#: Thirty-five consecutive patients with CP and CSM who underwent cervical operations between January 2006 and January 2014 were matched to 35 non-cerebral palsy (NCP) control patients. Postoperative complications and radiologic outcomes were compared between the groups. In the CP group, the Japanese Orthopaedic Association score; Oswestry neck disability index; modified Barthel index; and values for the grip and pinch, Box and Block, and Jebsen-Taylor hand function tests were obtained preand postoperatively and compared between those with and without postoperative complications. @*Results@#: Sixteen patients (16/35%) in the CP group and seven (7/35%) in the NCP group (p=0.021) had postoperative complications. Adjacent segment degeneration (p=0.021), postoperative motor weakness (p=0.037), and revisions (p=0.003) were significantly more frequent in the CP group than in the NCP group; however, instrument-related complications were not significantly higher in the CP group (7/35 vs. 5/35, p=0.280). The number of preoperative fixed cervical deformities were significantly higher in CP with postoperative complications (5/16 vs. 1/19, p=0.037). In the CP group, clinical outcomes were almost similar between those with and without postoperative complications. @*Conclusion@#: The occurrence of complications during the follow-up period was high in patients with CP. However, postoperative complications did not significantly affect clinical outcomes.

A Prospective, Multi-Center, Double-Blind, Randomized Study to Evaluate the Efficacy and Safety of the Synthetic Bone Graft Material DBM Gel with rhBMP-2 versus DBM Gel Used during the TLIF Procedure in Patients with Lumbar Disc Disease

Objective@#: This study is to evaluate the efficacy and safety of demineralized bone matrix (DBM) gel versus DBM gel with recombinant human bone morphogenetic protein-2 (rhBMP-2) used in transforaminal lumbar interbody fusion (TLIF). @*Methods@#: This study was designed as a prospective, multi-center, double-blind method, randomized study. All randomized subjects underwent TLIF with DBM gel with rhBMP-2 group (40 patients) as an experimental group or DBM gel group (36 patients) as a control group. Post-operative observations were performed at 12, 24, and 48 weeks. The spinal fusion rate on computed tomography scans and X-rays films, Visual analog scale pain scores, Oswestry disability index and SF-36 quality of life (QOL) scores were used for the efficacy evaluation. The incidence rate of adverse device effects (ADEs) and serious adverse device effects (SADEs) were used for safety evaluation. @*Results@#: The spinal fusion rate at 12 weeks for the DBM gel with rhBMP-2 group was higher with 73.68% compared to 58.82% for the DBM gel group. The 24 and 48 weeks were 72.22% and 82.86% for the DBM gel with rhBMP-2 group and 78.79% and 78.13%, respectively, for the DBM gel group. However, there were no significant differences between two groups in the spinal fusion rate at 12, 24, and 48 weeks post-treatment (p=0.1817, p=0.5272, p=0.6247). There was no significant difference between the two groups in the incidence rate of ADEs (p=0.3836). For ADEs in the experimental group, ‘Pyrexia’ (5.00%) was the most common ADE, followed by ‘Hypesthesia’, ‘Paresthesia’, ‘Transient peripheral paralysis’, ‘Spondylitis’ and ‘Insomnia’ (2.50%, respectively). ADEs reported in control group included ‘Pyrexia’, ‘Chest discomfort’, ‘Pain’, ‘Osteoarthritis’, ‘Nephropathy toxic’, ‘Neurogenic bladder’, ‘Liver function analyses’ and ‘Urticaria’ (2.86%, respectively). There was no significant difference between the two groups in the incidence rate of SADEs (p=0.6594). For SADE in the experimental group, ‘‘Pyrexia’ and ‘Spondylitis’ were 2.50%. SADE reported in the control group included 'Chest discomfort’, ‘Osteoarthritis’ and ‘Neurogenic bladder’. All SADEs described above were resolved after medical treatment. @*Conclusion@#: This study demonstrated that the spinal fusion rates of DBM gel group and DBM gel with rhBMP-2 group were not significantly different. But, this study provides knowledge regarding the earlier postoperative effect of rhBMP-2 containing DBM gel and also supports the idea that the longer term follow-up results are essential to confirm the safety and effectiveness.

Effect of Long-Term Treatment with Vagus Nerve Stimulation on Mood and Quality of Life in Korean Patients with Drug-Resistant Epilepsy

Background@#and Purpose This study aimed to determine the long-term effects of vagus nerve stimulation (VNS) treatment on suicidality, mood-related symptoms, and quality of life (QOL) in patients with drug-resistant epilepsy (DRE). We also investigated the relationships among these main effects, clinical characteristics, and VNS parameters. @*Methods@#Among 35 epilepsy patients who underwent VNS implantation consecutively in our epilepsy center, 25 patients were recruited to this study for assessing the effects of VNS on suicidality, mood-related symptoms, and QOL. The differences in these variables between before and after VNS treatment were analyzed statistically using paired t-tests. Multiple linear regression analyses were also performed to determine how the patients’ demographic and clinical characteristics influenced the variables that showed statistically significant changes after long-term VNS treatment. @*Results@#After VNS, our patients showed significant improvements not only in the mean seizure frequency but also in suicidality, depression, and QOL. The reduction in depression was associated with the improvement in QOL and more-severe depression at baseline. The reduction in suicidality was associated with higher suicidality at baseline, smaller changes in depression, and less-severe depression at baseline. Improved QOL was associated with lower suicidality at baseline. @*Conclusions@#This study found that VNS decreased the mean seizure frequency in patients with DRE, and also improved their depression, suicidality, and QOL. These results provide further evidence for therapeutic effect of VNS on psychological comorbidities of patients with DRE.

Preoperative Radiological Parameters to Predict Clinical and Radiological Outcomes after Laminoplasty

Many studies have focused on pre-operative sagittal alignment parameters which could predict poor clinical or radiological outcomes after laminoplasty. However, the influx of too many new factors causes confusion. This study reviewed sagittal alignment parameters, predictive of clinical or radiological outcomes, in the literature. Preoperative kyphotic alignment was initially proposed as a predictor of clinical outcomes. The clinical significance of the K-line and K-line variants also has been studied. Sagittal vertical axis, T1 slope (T1s), T1s-cervical lordosis (CL), anterolisthesis, local kyphosis, the longitudinal distance index, and range of motion were proposed to have relationships with clinical outcomes. The relationship between loss of cervical lordosis (LCL) and T1s has been widely studied, but controversy remains. Extension function, the ratio of CL to T1s (CL/T1s), and Sharma classification were recently proposed as LCL predictors. In predicting postoperative kyphosis, T1s cannot predict postoperative kyphosis, but a low CL/T1s ratio was associated with postoperative kyphosis.

Factors Affecting Postoperative Complications and Outcomes of Cervical Spondylotic Myelopathy with Cerebral Palsy : A Retrospective Analysis

Objective@#: Cervical surgery in patients with cervical spondylotic myelopathy (CSM) and cerebral palsy (CP) is challenging owing to the complexities of the deformity. We assessed factors affecting postoperative complications and outcomes after CSM surgery in patients with CP. @*Methods@#: Thirty-five consecutive patients with CP and CSM who underwent cervical operations between January 2006 and January 2014 were matched to 35 non-cerebral palsy (NCP) control patients. Postoperative complications and radiologic outcomes were compared between the groups. In the CP group, the Japanese Orthopaedic Association score; Oswestry neck disability index; modified Barthel index; and values for the grip and pinch, Box and Block, and Jebsen-Taylor hand function tests were obtained preand postoperatively and compared between those with and without postoperative complications. @*Results@#: Sixteen patients (16/35%) in the CP group and seven (7/35%) in the NCP group (p=0.021) had postoperative complications. Adjacent segment degeneration (p=0.021), postoperative motor weakness (p=0.037), and revisions (p=0.003) were significantly more frequent in the CP group than in the NCP group; however, instrument-related complications were not significantly higher in the CP group (7/35 vs. 5/35, p=0.280). The number of preoperative fixed cervical deformities were significantly higher in CP with postoperative complications (5/16 vs. 1/19, p=0.037). In the CP group, clinical outcomes were almost similar between those with and without postoperative complications. @*Conclusion@#: The occurrence of complications during the follow-up period was high in patients with CP. However, postoperative complications did not significantly affect clinical outcomes.

A Prospective, Multi-Center, Double-Blind, Randomized Study to Evaluate the Efficacy and Safety of the Synthetic Bone Graft Material DBM Gel with rhBMP-2 versus DBM Gel Used during the TLIF Procedure in Patients with Lumbar Disc Disease

Objective@#: This study is to evaluate the efficacy and safety of demineralized bone matrix (DBM) gel versus DBM gel with recombinant human bone morphogenetic protein-2 (rhBMP-2) used in transforaminal lumbar interbody fusion (TLIF). @*Methods@#: This study was designed as a prospective, multi-center, double-blind method, randomized study. All randomized subjects underwent TLIF with DBM gel with rhBMP-2 group (40 patients) as an experimental group or DBM gel group (36 patients) as a control group. Post-operative observations were performed at 12, 24, and 48 weeks. The spinal fusion rate on computed tomography scans and X-rays films, Visual analog scale pain scores, Oswestry disability index and SF-36 quality of life (QOL) scores were used for the efficacy evaluation. The incidence rate of adverse device effects (ADEs) and serious adverse device effects (SADEs) were used for safety evaluation. @*Results@#: The spinal fusion rate at 12 weeks for the DBM gel with rhBMP-2 group was higher with 73.68% compared to 58.82% for the DBM gel group. The 24 and 48 weeks were 72.22% and 82.86% for the DBM gel with rhBMP-2 group and 78.79% and 78.13%, respectively, for the DBM gel group. However, there were no significant differences between two groups in the spinal fusion rate at 12, 24, and 48 weeks post-treatment (p=0.1817, p=0.5272, p=0.6247). There was no significant difference between the two groups in the incidence rate of ADEs (p=0.3836). For ADEs in the experimental group, ‘Pyrexia’ (5.00%) was the most common ADE, followed by ‘Hypesthesia’, ‘Paresthesia’, ‘Transient peripheral paralysis’, ‘Spondylitis’ and ‘Insomnia’ (2.50%, respectively). ADEs reported in control group included ‘Pyrexia’, ‘Chest discomfort’, ‘Pain’, ‘Osteoarthritis’, ‘Nephropathy toxic’, ‘Neurogenic bladder’, ‘Liver function analyses’ and ‘Urticaria’ (2.86%, respectively). There was no significant difference between the two groups in the incidence rate of SADEs (p=0.6594). For SADE in the experimental group, ‘‘Pyrexia’ and ‘Spondylitis’ were 2.50%. SADE reported in the control group included 'Chest discomfort’, ‘Osteoarthritis’ and ‘Neurogenic bladder’. All SADEs described above were resolved after medical treatment. @*Conclusion@#: This study demonstrated that the spinal fusion rates of DBM gel group and DBM gel with rhBMP-2 group were not significantly different. But, this study provides knowledge regarding the earlier postoperative effect of rhBMP-2 containing DBM gel and also supports the idea that the longer term follow-up results are essential to confirm the safety and effectiveness.

Effect of Long-Term Treatment with Vagus Nerve Stimulation on Mood and Quality of Life in Korean Patients with Drug-Resistant Epilepsy

Background@#and Purpose This study aimed to determine the long-term effects of vagus nerve stimulation (VNS) treatment on suicidality, mood-related symptoms, and quality of life (QOL) in patients with drug-resistant epilepsy (DRE). We also investigated the relationships among these main effects, clinical characteristics, and VNS parameters. @*Methods@#Among 35 epilepsy patients who underwent VNS implantation consecutively in our epilepsy center, 25 patients were recruited to this study for assessing the effects of VNS on suicidality, mood-related symptoms, and QOL. The differences in these variables between before and after VNS treatment were analyzed statistically using paired t-tests. Multiple linear regression analyses were also performed to determine how the patients’ demographic and clinical characteristics influenced the variables that showed statistically significant changes after long-term VNS treatment. @*Results@#After VNS, our patients showed significant improvements not only in the mean seizure frequency but also in suicidality, depression, and QOL. The reduction in depression was associated with the improvement in QOL and more-severe depression at baseline. The reduction in suicidality was associated with higher suicidality at baseline, smaller changes in depression, and less-severe depression at baseline. Improved QOL was associated with lower suicidality at baseline. @*Conclusions@#This study found that VNS decreased the mean seizure frequency in patients with DRE, and also improved their depression, suicidality, and QOL. These results provide further evidence for therapeutic effect of VNS on psychological comorbidities of patients with DRE.

Surgical Strategy for Sacral Tumor Resection

Purpose@#This study aimed to present our experiences with a precise surgical strategy for sacrectomy. @*Materials and Methods@#This study comprised a retrospective review of 16 patients (6 males and 10 females) who underwent sacrectomy from 2011 to 2019. The average age was 42.4 years old, and the mean follow-up period was 40.8 months. Clinical data, including age, sex, history, pathology, radiographs, surgical approaches, onset of recurrence, and prognosis, were analyzed. @*Results@#The main preoperative symptom was non-specific local pain. Nine patients (56%) complained of bladder and bowel symptoms. All patients required spinopelvic reconstruction after sacrectomy. Three patients, one high, one middle, and one hemisacrectomy, underwent spinopelvic reconstruction. The pathology findings of tumors varied (chordoma, n=7; nerve sheath tumor, n=4; giant cell tumor, n=3, etc.). Adjuvant radiotherapy was performed for 5 patients, chemotherapy for three, and combined chemoradiotherapy for another three. Six patients (38%) reported postoperative motor weakness, and newly postoperative bladder and bowel symptoms occurred in 5 patients. Three patients (12%) experienced recurrence and expired. @*Conclusion@#In surgical resection of sacral tumors, the surgical approach depends on the size, location, extension, and pathology of the tumors. The recommended treatment option for sacral tumors is to remove as much of the tumor as possible. The level of root sacrifice is a predicting factor for postoperative neurologic functional impairment and the potential for morbidity. Pre-operative angiography and embolization are recommended to prevent excessive bleeding during surgery. Spinopelvic reconstruction must be considered following a total or high sacrectomy or sacroiliac joint removal.

Effects of an Educational Program Based on Mobile SMS and Counseling for Colonoscopy in the Elderly

Purpose@#This study was conducted to examine the effects of an educational program based on mobile SMS and counseling for colonoscopy in elderly people. Methods: The study utilized a non-equivalent control group with a non-synchronized design as a quasiexperimental research. The study subjects consisted of 72 elderly people (36 elderly people in the experimental group and 36 in the control group). Among the subjects, 49 were male (68.1%) and 23 were female (31.9%), and the age was 69.04± 6.14 years. Fortyfive subjects (62.5%) answered that they had underlying diseases, eight subjects (11.1%) had a family history of colon disease, and 61 subjects (84.7%) had undergone a colonoscopy. The experimental group was provided with an educational program based on mobile SMS and counseling when three days and one day before the test. The control group received verbal explanations using information sheet. The data were analyzed using descriptive statistics, the Chi-squares test, and the independent t-test with SPSS/WIN 21.0. @*Results@#Significant differences were found in bowel cleanliness scores (t= 3.60, p= .003) between the groups. No significant differences were seen between the groups in the sedation drug dosage (propofol (t= 1.02, p= .730), midazolam (t= 0.66, p= .380)) and time for colonoscopy (t= 1.08, p= .235). Conclusion: Based on the results of this study, an educational program based on mobile SMS and counseling for colonoscopy was proposed as an educational program before colonoscopy.

A patient with multiple arterial stenosis diagnosed with Alagille syndrome: A case report

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

Left atrial wall thickness and its relationship with reconnection after pulmonary vein isolation in patients with atrial fibrillation evaluated using a three‑dimensional wall thickness map

Background@#The major cause of recurrence after pulmonary vein (PV) isolation for atrial fibrillation (AF) is PV recon‑ nection, and thicker wall could be associated with reconnection. @*Objectives@#This study aimed to evaluate the wall thickness of the PV antrum in reconnection sites using a threedimensional (3D) wall thickness map. @*Methods@#A total of 91 patients who underwent a second ablation procedure due to AF recurrence were evalu‑ ated. The locations of the PV reconnection sites were confirmed in electroanatomical maps. A 3D atrial wall thickness (AWT) map was created using computed tomography scan data. The AWT values of the ablation lines of the index procedure were graded in each segment of the PV antrum: grade 1, 0.5 2.5 mm. @*Results@#A total of 281 PV reconnection sites among 1256 segments of the PV antrum in 79 patients were detected. The average AWT grades were 2.7 ± 1.0 and 2.2 ± 1.0 in the reconnected and non-reconnected segments, respectively (P < 0.01). Higher AWT grades were observed in the reconnected superior segments of the left superior PV, carina and inferior segments of the left inferior PV, superior and posterior segments of the right superior PV, and posterior and inferior segments of the right inferior PV. @*Conclusion@#The reconnected segments of the PV antrum showed thicker myocardium than the non-reconnected ones in patients with recurrent AF after catheter ablation. A wall thickness map for PV isolation could be considered for customized ablation in order to reduce PV reconnection.

A case report of recurrent anaphylaxis in a 36-year-old female:Lipid transfer protein syndrome

Lipid transfer protein (LTP) is a widely cross-reacting allergen in vegetables, fruits, nuts, or cereals. The amino acids sequence of LTP shows high structural similarity in various foods and thus acts as a pan-allergen. LTP syndrome is a new term for the pattern of clinical symptoms and natural course of allergy caused by LTP antigens. It is frequently reported by peaches mainly in the Mediterranean region, and reports in East Asia are rare. Therefore, this case report introduces a 36-year-old female Asian who experienced recurrent anaphylaxis and oral allergy syndrome, and finally diagnosed with LTP syndrome.

Combined Method of Neuronal Cell-Inducible Vector and Valproic Acid for Enhanced Gene Expression under Hypoxic Conditions

Background@#Gene therapy shows the ability to restore neuronal dysfunction via therapeutic gene expression. The efficiency of gene expression and delivery to hypoxic injury sites is important for successful gene therapy. Therefore, we established a gene/stem cell therapy system using neuron-specific enolase promoter and induced neural stem cells in combination with valproic acid to increase therapeutic gene expression in hypoxic spinal cord injury. @*Methods@#To examine the effect of combined method on enhancing gene expression, we compared neuronal cell-inducible luciferase levels under normoxia or hypoxia conditions in induced neural stem cells with valproic acid. Therapeutic gene, vascular endothelial growth factor, expression with combined method was investigated in hypoxic spinal cord injury model. We verified gene expression levels and the effect of different methods of valproic acid administration in vivo. @*Results@#The results showed that neuron-specific enolase promoter enhanced gene expression levels in induced neural stem cells compared to Simian Virus 40 promoter under hypoxic conditions. Valproic acid treatment showed higher gene expression of neuron-specific enolase promoter than without treatment. In addition, gene expression levels and cell viability were different depending on the various concentration of valproic acid. The gene expression levels were increased significantly when valproic acid was directly injected with induced neural stem cells in vivo. @*Conclusion@#In this study, we demonstrated that the combination of neuron-specific enolase promoter and valproic acid induced gene overexpression in induced neural stem cells under hypoxic conditions and also in spinal cord injury depending on valproic acid administration in vivo. Combination of valproic acid and neuron-specific enolase promoter in induced neural stem cells could be an effective gene therapy system for hypoxic spinal cord injury.

Hypotonia, Ataxia, and Delayed Development Syndrome caused by the EBF3 mutation in a Korean boy with muscle hypotonia

Hypotonia, Ataxia, and Delayed Development Syndrome (HADDS) is an autosomal-dominant, extremely rare neurodevelopmental disorder caused by the heterozygous EBF3 gene mutation. EBF3 is located on chromosome 10q26.3 and acts as a transcription factor that regulates neurogenesis and differentiation. This syndrome is characterized by dysmorphism, cerebellar hypoplasia, urogenital anomaly, hypotonia, ataxia, intellectual deficit, and speech delay. The current report describes a 3-year-old Korean male carrying a de novo EBF3 mutation, c.589A>G (p.Asn197Asp), which was identified by whole exome sequencing. He manifested facial dysmorphism, hypotonia, strabismus, vermis hypoplasia, and urogenital anomalies, including vesicoureteral reflux, cryptorchidism, and areflexic bladder. This is the first report of a case of HADDS cause by an EBF3 mutation in the Korean population.

Surgical Outcomes of Dysphagia Provoked by Diffuse Idiopathic Skeletal Hyperostosis in the Cervical Spine

PURPOSE: This study aimed to predict the surgical outcomes of diffuse idiopathic skeletal hyperostosis (DISH)-related dysphagia (DISH-phagia) and to evaluate the importance of prevertebral soft tissue thickness (PVST).MATERIALS AND METHODS: In total, 21 surgeries (anterior osteophytectomy or anterior cervical decompression and fixation) were included in this study for DISH-phagia from 2003 to 2019. Clinical outcomes were assessed using the Dysphagia Outcome and Severity Scale (DOSS) preoperatively, at 1 month postoperatively, and last follow up (mean 29.5 months). PVST was measured using lateral plain radiographs. Paired t-test and Spearman's correlation test was used to identify relationships between various PVST indices and DOSS.RESULTS: Comparisons were made from 17 patients out of 21, in which the record had all of three measurements. The narrowest PVST preoperatively was 2.55±0.90 mm, with a DOSS score of 4.47±1.61, and that at 1 month after surgery was 5.02±2.33 mm, with a DOSS score of 6.12±1.32. At last follow up, PVST and DOSS values were 3.78±0.92 mm and 5.82±1.34, and three patients experienced symptom relapse. Significant relationships were found between PVST and DOSS at all time points: before surgery (R=0.702, p<0.001), 1 month after surgery (R=0.539, p=0.012), and last follow up (R=0.566, p=0.020).CONCLUSION: Surgical removal of anterior osteophytes is an effective treatment option for DISH-phagia, and PVST is a useful parameter in DISH-phagia. The goal of DISH surgery should be to remove DISH as much as possible to ensure sufficient PVST postoperatively.

Combination Therapy by Tissue-Specific Suicide Gene and Bevacizumab in Intramedullary Spinal Cord Tumor

Purpose@#Malignant gliomas are aggressive spinal cord tumors. In this study, we hypothesized that combination therapy using an anti-angiogenic agent, bevacizumab, and hypoxia-inducible glioblastoma-specific suicide gene could reduce tumor growth. @*Materials and Methods@#In the present study, we evaluated the effect of combination therapy using bevacizumab and pEpo-NI2-SV-TK in reducing the proliferation of C6 cells and tumor growth in the spinal cord. Spinal cord tumor was generated by the injection of C6 cells into the T5 level of the spinal cord. Complexes of branched polyethylenimine (bPEI)/pEpo-NI2-SV-TK were injected into the spinal cord tumor. Bevacizumab was then administered by an intraperitoneal injection at a dose of 7 mg/kg. The anti-cancer effects of combination therapy were analyzed by histological analyses and magnetic resonance imaging (MRI). The Basso, Beattie and Bresnahan scale scores for all of the treatment groups were recorded every other day for 15 days to assess the rat hindlimb strength. @*Results@#The complexes of bPEI/pEpo-NI2-SV-TK inhibited the viability of C6 cells in the hypoxia condition at 5 days after treatment with ganciclovir. Bevacizumab was decreased in the cell viability of human umbilical vein endothelial cells. Combination therapy reduced the tumor size by histological analyses and MRI. The combination therapy group showed improved hind-limb function compared to the other groups that were administered pEpo-NI2-SV-TK alone or bevacizumab alone. @*Conclusion@#This study suggests that combination therapy using bevacizumab with the pEpo-NI2-SV-TK therapeutic gene could be useful for increasing its therapeutic benefits for intramedullary spinal cord tumors.