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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the degeneration of upper motor neurons in the brainstem and spinal cord and lower motor neurons. ALS was first described by Jean-Martin Charcot in 1874 based on clinical features and postmortem examinations. In 1990, the first diagnostic criteria for ALS were developed based on clinical features. Subsequently, three additional diagnostic criteria were published. In this article, we introduce the clinical features, diagnostic criteria, and diseases that need to be differentiated in ALS.
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Purpose@#The objective of the study was to explore how urology-related news, one of the medical specialties profoundly linked to human health and life, is communicated to the public through media outlets that serves as primary sources of medical information for the public. @*Methods@#In this study, articles were retrieved using the keyword ‘Urology’ from the Bigkinds spanning from January 1, 1990 to August 17, 2023. The Beautifulsoup library in Python was utilized for parsing the text to collect both titles and bodies of the articles. The gathered data was then analyzed using the latent Dirichlet allocation (LDA) algorithm from the scikit-learn library. Additionally, tools such as Wordcloud and Networkx were employed to visualize the relationships and patterns within the data. @*Results@#The keyword analysis led to the identification of various themes in the articles, with a clear distinction between those providing medical information and those promoting healthcare services, technologies, and products. Notably, the content frequently intertwined informational aspects with promotional ones. Articles on men’s health and pet diseases, for example, often combined educational material with product or procedure promotions. This overlap highlights the complexity of categorizing media content into distinct themes. Furthermore, the coverage of health insurance and treatment methods including recent advancements like robotic surgery reflected the evolving nature of healthcare discussions to emphasize the interplay between policy changes, medical advancements, and media portrayal. @*Conclusions@#By identifying 10 distinct topics mentioned in the news, the analysis determined which topics are common in urology-related news coverage. The findings revealed a substantial volume of medical information on urology in the media with a wide range of topics including treatment and prevention of urologic conditions, insurance information, new treatments, and news stories promoting new products or hospitals.
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Objective@#Dysphagia is a common symptom of stroke and affects 23–50% of such patients. In addition, bulbar involvement, which causes dysphagia, is the primary initial symptom in approximately 25–30% of amyotrophic lateral sclerosis (ALS) patients. The purpose of this study was to compare patterns of swallowing difficulties in stroke and ALS patients. @*Methods@#We retrospectively recruited 84 ALS patients with dysphagia and 294 stroke patients with dysphagia between January 2017 and December 2019. Swallowing processes were reviewed by videofluoroscopic swallowing studies (VFSSs). The presence of oral residues and oral transit times (OTTs) were measured in the oral phase, and the presence of penetration and aspiration and residues in valleculae or pyriform sinuses were evaluated. Statistical analysis was performed using SPSS 25.0 and comparisons using the Chi-square test. @*Results@#ALS patients more frequently had delayed OTTs and oral residues than stroke patients, and stroke patients more frequently experienced aspiration and had delayed thin liquid pharyngeal transit times (PTTs). However, no significant intergroup difference was observed for the presence of penetration, residues in valleculae or pyriform sinuses, or thick liquid PTTs. @*Conclusion@#The study shows that ALS patients exhibit slower food processing in the oral cavity and more significant bulbar muscle weakness than stroke patients. On the other hand, stroke patients had greater thin liquid aspiration rates than ALS patients. These findings should be considered when choosing treatments for ALS and stroke.
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Background@#Intermaxillary fixation (IMF) is a technique that allows for the reduction and stabilization of mandibular fractures. Several methods of IMF, such as self-tapping screws or arch bars, have been developed. This study aimed to validate the usefulness of IMF with a self-tapping screw compared to IMF with arch bars with focus on the patients’ perspective. @*Methods@#We retrospectively reviewed the medical records of all patients who were treated for mandibular fractures at our hospital between August 2014 and February 2021. A total of 57 patients were enrolled in this study. Thirteen patients were excluded from the analysis: three patients were lost to follow-up, and 10 patients did not undergo IMF. Finally, 44 patients were analyzed, of which 31 belonged to the arch bar group, and 13 belonged to the screw group. Patient discomfort and pain during IMF application and removal were analyzed using a patient self-assessment questionnaire. The surgeon also assessed oral hygiene, IMF stability, and occlusion. @*Results@#We applied IMF to 34 men (77%) and 10 women (23%). The mean age of the patients was 37.3 years. The most common fracture site was the angle (30%), followed by the parasymphysis (25%), the body (23%), the condyle (11%), and the ramus (11%). Patient discomfort and oral hygiene were statistically favorable in the screw group. The IMF application time was statistically shorter in the screw group (p< 0.001). IMF stability was not statistically different between the two groups. The pain score during IMF removal was lower in the screw group (p< 0.001). @*Conclusion@#Compared to arch bars, IMF screws provide more comfort during the IMF period, help maintain favorable oral hygiene, and have a shorter application time. From the patient’s perspective, IMF screws are an excellent alternative to conventional arch bars when applicable.
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Background@#Xanthelasma palpebrarum (XP) is a benign periorbital lesion. The relationship between xanthelasma lesion size and serum cholesterol levels has been poorly studied. In this study, we investigated this relationship in the context of the clinical etiology of XP. Methods: We retrospectively reviewed medical records and pathology reports of all patients treated for XP at our hospital between June 2014 and June 2021; the data were used to analyze lesion size, underlying disease, serum cholesterol, and disease recurrence. @*Results@#The mean values for patient age, serum cholesterol, and lesion size were 53.0 years, 235.0 mg/dL, and 69.2 mm2, respectively. XP mainly occurred in women (64.7%); furthermore, the incidence of XP and lesion size was greatest among patients in their 5th decade of life (41.2%). There was no statistically significant relationship between xanthelasma lesion size and serum cholesterol level. @*Conclusion@#This study compared lesion size with various clinical features in XP patients. In patients who underwent surgery for XP, serum cholesterol levels tended to be higher than those in the general population. However, the trend between the size of XP and serum cholesterol level was unclear. Therefore, if a patient with XP visits the hospital for surgery, it is recommended to check the lipid profile to confirm underlying dyslipidemia regardless of the size.
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Background@#Nasal septal cartilage is used to obtain favorable aesthetic and functional outcomes in rhinoplasty, but is often difficult to harvest or the harvested amount is insufficient. Therefore, the objective of this study is to introduce how to harvest septal cartilage optimally without losing and use harvested cartilage efficiently. @*Methods@#From March 2015 to January 2020, we tried to harvest as much septal cartilage as possible while maintaining the L-strut in 30 patients. A spreader flap and septal rotation suture were used instead of a spreader graft. Also in patients who needed a spreader graft and septal extension graft, a spreader graft was used on one side and a one-piece spreader graft combined with a septal extension graft was performed on the other side. For tip plasty, a columella septal suture was performed first. Postoperative patient satisfaction was assessed using the Rhinoplasty Outcome Examination questionnaire. @*Results@#No serious complications were observed. The patient satisfaction score was 50% or above in 27 patients (90%) and less than 50% in only three patients (10%). The average score was 81.5 points. @*Conclusion@#For septal cartilage deficiency, a spreader flap, the septal rotation suture, or onepiece spreader graft combined with a septal extension graft was used. The nasal tip was sufficiently rotated using the columellar septal suture technique first. These techniques made it possible to obtain good aesthetic outcomes using only septal cartilage, without harvesting other cartilage.
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Background@#Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron degeneration with phenotypic heterogeneity, including age at onset. Juvenile ALS (JALS) includes ALS patients aged less than 25 years who typically show slow progression. This study aimed to identify the characteristics of juvenile ALS from Korean ALS cohorts. @*Methods@#We retrospectively investigated the clinical characteristics of JALS patients, who met the revised El Escorial-Airlie House criteria, in the Korean motor neuron disease cohort om January 2002 to November 2018. To evaluate the genetic background ofin JALS, we screened the SOD1 mutation in all JALS patients using PCR. @*Results@#Among the seven JALS patients, the mean age was 22.1 years (± 2.19 years) and 4 patients were male. Most patients were diagnosed within less than 12 months, but in one patient, it took 96 months to make the initial diagnosis. On assessing the cognitive function, none of the patients had dementia. The progression rate of JALS during follow-up was usually low (median [IQR], 0.31 [0.11-0.52]), except in patients with SOD1 mutation (3.40) and CLEC4C mutation (1.12). One patient revealed a family history of ALS with SOD1 mutation, but we also detected the SOD1 mutation among sporadic JALS patients. @*Conclusions@#Although JALS patients with genetic mutations (SOD1-p.Asn87Ser and CLEC4C-p.Lys210*) showed faster progression than other JALS patients, one patient with SOD1 mutation (p.Gly17Ala) showed slow progression. Familial ALS was rare; however, it might be caused by low or incomplete penetrance of the genes or by small number of JALS patients. To investigate the other genetic causes of JALS without the SOD1 mutation, a further study including detailed genetic analysis is needed.
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Schwannomas, which originate from Schwann cells in the peripheral nervous system, are slowgrowing and uncommon benign tumors. Most schwannomas (90%) occur in isolation, and multiple occurrences are a characteristic feature of neurofibromas. Schwannomas of the nose and nasal tip are particularly unusual. Although a few cases of schwannomas of the sinusoidal tract and nasal septum have been reported, schwannomas arising from the nasal dorsum area and tip are extremely rare. Sensory abnormalities are also a very rare symptom. We excised a schwannoma on the nasal dorsum through direct incision and a schwannoma on the nasal tip through open rhinoplasty. No postoperative complications involving recurrence, hematoma, or infection occurred. The possibility of neurological changes should be considered in cases of an abnormality in the peripheral nervous system. Schwannoma must be kept in mind as a possible cause of neurological changes localized to a specific dermatome, and should always be considered in the differential diagnosis of a mass on the nose.
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Purpose@#The current standard chemotherapy for advanced biliary tract cancer (BTC) has limited benefit,and novel therapies need to be investigated. @*Materials and Methods@#In this prospective cohort study, programmed death ligand-1 (PD-L1)–positive BTC patientswho progressed on first-line gemcitabine plus cisplatin were enrolled. Pembrolizumab 200mg was administered intravenously every 3 weeks. @*Results@#Between May 2018 and February 2019, 40 patients were enrolled. Pembrolizumab wasgiven as second-line (47.5%) or third-line therapy (52.5%). The objective response ratewas 10% and 12.5% by Response Evaluation Criteria in Solid Tumor (RECIST) v1.1 andimmune-modified RECIST (imRECIST) and median duration of response was 6.3 months.Among patients with progressive disease as best response, one patient (1/20, 5.0%)achieved complete response subsequently. The median progression-free survival (PFS) andoverall survival (OS) were 1.5 months (95% confidence interval [CI], 0.0 to 3.0) and 4.3months (95% CI, 3.5 to 5.1), respectively, and objective response per imRECIST was significantlyassociated with PFS (p < 0.001) and OS (p=0.001). Tumor proportion score 50%was significantly associated with higher response rates including the response after pseudoprogression(vs. < 50%; 37.5% vs. 6.5%; p=0.049). @*Conclusion@#Pembrolizumab showed modest anti-tumor activity in heavily pretreated PD-L1–positiveBTC patients. In patients who showed objective response, durable response could beachieved.
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Epidermoid cysts are benign tumors that account for approximately 1% of intracranial tumors. Invery rare cases, temporally located extradural intradiploic epidermoid cysts can cause neurologicalsymptoms and skull perforation. Herein, we report the case of a 34-year-old woman who underwentsuccessful treatment of an epidermoid cyst in the temporal region accompanied by neurologicalsymptoms. Accurate radiological evaluation and complete removal of the tumor and capsuleplay a vital role in ensuring favorable long-term outcomes. Computed tomography and magneticresonance imaging scans can provide an accurate assessment of the extent of intracranialexpansion and invasion of the cerebral parenchyma, as well as enabling the precise localizationand characterization of the bone defect and mass. In addition, collaborative surgery with a neurosurgeonis required for cases involving intracranial expansion and dural invasion.
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Objective@#To review trends in bladder emptying methods over a 20-year period in patients with spinal cord injury (SCI) by severity according to the American Spinal Injury Association impairment scale (AIS). @*Methods@#Medical records of patients with SCI from 1994 to 1998 (group 1) and from 2012 to 2016 (group 2) were retrospectively reviewed. We classified bladder emptying methods according to the International Spinal Cord dataset. We grouped patients with normal voiding, bladder reflex triggering, and bladder expression as those using voiding without catheter. @*Results@#A total of 667 patients were included in the analysis. The proportion of patients using voiding without catheter and intermittent catheterization decreased from 67.0% to 30.0% and increased from 26.8% to 54.8%, respectively. In patients with AIS-A and AIS-B, the proportion of patients with intermittent catheterization increased from 32.8% to 73.3%. In patients with AIS-D, the proportion of patients using voiding without catheter and intermittent catheterization decreased from 88.5% to 68.9% and increased from 11.5% to 26.8%, respectively. In group 2, among 111 patients with AIS-D using voiding without catheter at admission, 8 (7.2%) switched to intermittent catheterization at discharge due to decreased bladder volume, increased post-voiding residual urine, or incontinence. @*Conclusion@#Over the past 20 years, trends in bladder emptying methods in patients with SCI changed from voiding without catheter to intermittent catheterization in Korea. This was especially prominent in patients with AIS-A, AIS-B, and AIS-C. Even in patients with AIS-D, the use of intermittent catheterization at hospital discharge increased.
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Objective@#To translate the Stroke Rehabilitation Motivation Scale (SRMS), developed to evaluate the motivation level of stroke patients during rehabilitation, into the Korean language and to verify the reliability and validity of the Korean version of SRMS (K-SRMS). @*Methods@#The K-SRMS was developed following a structured process that included translation, verification, compromise assessment, reverse translation, feedback, and final correction. K-SRMS reliability was evaluated by performing internal consistency and test–retest analyses. The reliability test was conducted in 50 stroke patients. Its validity was assessed by comparing the K-SRMS with the scale and performing exploratory factor analysis. The validity test was conducted in 102 stroke patients. @*Results@#The test–retest analysis showed good reliability, and the internal consistency of the K-SRMS was similar to that of the original version for all, except 4, items. Thus, these 4 items were excluded, and then the validity test was conducted. Pearson correlation analysis demonstrated that the K-SRMS score was significantly correlated with the BAS total score (Pearson r=0.207, p<0.05). In the exploratory factor analysis, K-SRMS items were categorized into 7 groups (factors), and factors 1 and 4 showed mutual concordance with K-SRMS subscales, including intrinsic motivation factors and amotivation, respectively. @*Conclusion@#The newly developed K-SRMS showed good reliability and validity. It could also be used as a tool to objectify the degree of motivation for rehabilitation among stroke patients in clinical care and research.
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Objective@#To compare the energy efficiency of gait with knee-ankle-foot orthosis (KAFO) and robot-assisted gait and to develop a usability questionnaire to evaluate the satisfaction of walking devices in paraplegic patients with spinal cord injuries. @*Methods@#Thirteen patients with complete paraplegia participated and 10 completed the evaluation. They were trained to walk with KAFO (KAFO-gait) or a ReWalk robot (ReWalk-gait) for 4 weeks (20 sessions). After a 2-week wash-out period, they switched walking devices and underwent 4 additional weeks of training. Two evaluations were performed (after 2 and 4 weeks) following the training periods for each walking device, using the 6-minute walking test (6MWT) and 30-minute walking test (30MWT). The spatiotemporal variables (walking distance, velocity, and cadence) and energy expenditure (heart rate, maximal heart rate, the physiologic cost index, oxygen consumption, metabolic equivalents, and energy efficiency) were evaluated duringthe 6MWT and 30MWT. A usability evaluation questionnaire for walking devices was developed based on the International Organization for Standardization/International Electrotechnical Commission guidelines through expert consultation. @*Results@#The ReWalk-gait presented significant advantages in energy efficiency compared to KAFO-gait in the 6MWT and 30MWT; however, there were no differences in walking distance or speed in the 30MWT between ReWalk-gait and KAFOgait. The usability test demonstrated that ReWalk-gait was not superior to KAFO-gait in terms of safety, efficacy, efficiency, or patient satisfaction. @*Conclusion@#The robot (ReWalk) enabled patients with paraplegia to walk with lower energy consumption compared to KAFO, but the ReWalk-gait was not superior to KAFO-gaitin terms of patient satisfaction.
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Background@#Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron degeneration with phenotypic heterogeneity, including age at onset. Juvenile ALS (JALS) includes ALS patients aged less than 25 years who typically show slow progression. This study aimed to identify the characteristics of juvenile ALS from Korean ALS cohorts. @*Methods@#We retrospectively investigated the clinical characteristics of JALS patients, who met the revised El Escorial-Airlie House criteria, in the Korean motor neuron disease cohort om January 2002 to November 2018. To evaluate the genetic background ofin JALS, we screened the SOD1 mutation in all JALS patients using PCR. @*Results@#Among the seven JALS patients, the mean age was 22.1 years (± 2.19 years) and 4 patients were male. Most patients were diagnosed within less than 12 months, but in one patient, it took 96 months to make the initial diagnosis. On assessing the cognitive function, none of the patients had dementia. The progression rate of JALS during follow-up was usually low (median [IQR], 0.31 [0.11-0.52]), except in patients with SOD1 mutation (3.40) and CLEC4C mutation (1.12). One patient revealed a family history of ALS with SOD1 mutation, but we also detected the SOD1 mutation among sporadic JALS patients. @*Conclusions@#Although JALS patients with genetic mutations (SOD1-p.Asn87Ser and CLEC4C-p.Lys210*) showed faster progression than other JALS patients, one patient with SOD1 mutation (p.Gly17Ala) showed slow progression. Familial ALS was rare; however, it might be caused by low or incomplete penetrance of the genes or by small number of JALS patients. To investigate the other genetic causes of JALS without the SOD1 mutation, a further study including detailed genetic analysis is needed.
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Schwannomas, which originate from Schwann cells in the peripheral nervous system, are slowgrowing and uncommon benign tumors. Most schwannomas (90%) occur in isolation, and multiple occurrences are a characteristic feature of neurofibromas. Schwannomas of the nose and nasal tip are particularly unusual. Although a few cases of schwannomas of the sinusoidal tract and nasal septum have been reported, schwannomas arising from the nasal dorsum area and tip are extremely rare. Sensory abnormalities are also a very rare symptom. We excised a schwannoma on the nasal dorsum through direct incision and a schwannoma on the nasal tip through open rhinoplasty. No postoperative complications involving recurrence, hematoma, or infection occurred. The possibility of neurological changes should be considered in cases of an abnormality in the peripheral nervous system. Schwannoma must be kept in mind as a possible cause of neurological changes localized to a specific dermatome, and should always be considered in the differential diagnosis of a mass on the nose.
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Guillain-Barre syndrome (GBS) is acute inflammatory demyelinating polyradiculoneuropathy, which is often related to post-infectious etiology. However, GBS has also been reported to be caused by non-infectious factors such as trauma. This report describes a rare case of post-traumatic GBS with dramatic response to immunoglobulin therapy. And here, we also discussed about the importance of differential diagnosis with critical illness polyneuropathy.
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Alzheimer's disease (AD), the most common form of dementia, has emerged as a major global public health challenge. However, the complexity of AD in its biological, genetic, and clinical aspects has hindered the development of effective therapeutic agents. Research plans that integrate new drug discoveries are urgently needed, including those based on novel and reliable biomarkers that reflect not only clinical phenotype, but also genetic and neuroimaging information. Therapeutic strategies such as stratification (i.e., subgrouping of patients having similar clinical characteristics or genetic background) and personalized medicine could be set as new directions for developing effective drugs for AD. In this review, we describe a therapeutic strategy that is based on immune-inflammation modulation for a subgroup of AD and related dementias, arguing that the use of stratification and personalized medicine is a promising way to achieve targeted medicine. The Korean AD Research Platform Initiative based on Immune-Inflammatory biomarkers (K-ARPI) has recently launched a strategy to develop novel biomarkers to identify a subpopulation of patients with AD and to develop new drug candidates for delaying the progression of AD by modulating toxic immune inflammatory response. Sphingosine kinase 1 (SphK1) and its metabolites, triggering receptor expressed on myeloid cells-2 (TREM2) related signals, and actin motility related proteins including Nck-associated protein 1 (Nap1) were selected as promising targets to modulate neuroinflammation. Their roles in stratification and personalized medicine will be discussed.
Subject(s)
Humans , Actins , Alzheimer Disease , Biomarkers , Dementia , Inflammation , Neuroimaging , Phenotype , Phosphotransferases , Precision Medicine , Public Health , SphingosineABSTRACT
BACKGROUND: Tuberculosis (TB) is a major infectious disease in South Korea causing substantial disease burden, particularly in the elderly. This study aimed to identify the case detection rate of mobile TB screening for the elderly conducted in the Jeollanam-do region and to analyze risk factors of active TB. METHODS: We screened the elderly population (≥65 years old) in Jeollanam-do from August to December 2017. Chest radiography was performed for all participants. Participants with TB presumptive signs were asked to submit sputum specimen(s). Sputum smear, culture, and polymerase chain reaction analyses were performed. Cascade analysis, chi-squared tests, and Fisher exact tests were used to evaluate screening performance. RESULTS: In total, 12,402 participants were screened, and 211 (1.7%) were suspected to have active TB; 181 of the suspected patients (85.8%) underwent sputum smear test, and 16 (8.8%) patients were confirmed to have TB. The TB prevalence among the elderly was bacteriologically confirmed to be 129 per 100,000 individuals, which was similar to national TB notification data for the same age groups. The proportion of active TB cases increased with age, and differed based on sex and past TB history. However, TB-related symptoms, comorbidity status, and TB screening history within 12 months were not predictive of active TB. CONCLUSION: This study identified that the prevalence rate was similar to national TB notification data from the same age groups. Periodic, community-based, systematic TB screening among the elderly population is recommended.
Subject(s)
Aged , Humans , Asian People , Communicable Diseases , Comorbidity , Korea , Mass Screening , Pilot Projects , Polymerase Chain Reaction , Prevalence , Radiography , Risk Factors , Sputum , Thorax , TuberculosisABSTRACT
Orocutaneous fistulas, or cutaneous sinuses of odontogenic origin, are uncommon but often misdiagnosed as skin lesions unrelated to dental origin by physicians. Accurate diagnosis and use of correct investigative modalities are important because orocutaneous fistulas are easily confused for skin or bone tumors, osteomyelitis, infected cysts, salivary gland fistulas, and other pathologies. The aim of this study is to present our experience with a patient with orocutaneous fistulas of odontogenic origin presenting as recurrent pyogenic granuloma of the cheek, and to discuss their successful treatment.
Subject(s)
Humans , Cheek , Cutaneous Fistula , Diagnosis , Fistula , Granuloma, Pyogenic , Osteomyelitis , Pathology , Recurrence , Salivary Gland Fistula , SkinABSTRACT
BACKGROUND@#Tuberculosis (TB) is a major infectious disease in South Korea causing substantial disease burden, particularly in the elderly. This study aimed to identify the case detection rate of mobile TB screening for the elderly conducted in the Jeollanam-do region and to analyze risk factors of active TB.@*METHODS@#We screened the elderly population (≥65 years old) in Jeollanam-do from August to December 2017. Chest radiography was performed for all participants. Participants with TB presumptive signs were asked to submit sputum specimen(s). Sputum smear, culture, and polymerase chain reaction analyses were performed. Cascade analysis, chi-squared tests, and Fisher exact tests were used to evaluate screening performance.@*RESULTS@#In total, 12,402 participants were screened, and 211 (1.7%) were suspected to have active TB; 181 of the suspected patients (85.8%) underwent sputum smear test, and 16 (8.8%) patients were confirmed to have TB. The TB prevalence among the elderly was bacteriologically confirmed to be 129 per 100,000 individuals, which was similar to national TB notification data for the same age groups. The proportion of active TB cases increased with age, and differed based on sex and past TB history. However, TB-related symptoms, comorbidity status, and TB screening history within 12 months were not predictive of active TB.@*CONCLUSION@#This study identified that the prevalence rate was similar to national TB notification data from the same age groups. Periodic, community-based, systematic TB screening among the elderly population is recommended.