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Ataxia is presented by various etiologies, including acquired, genetic and degenerative disorders. Although hereditary ataxia is suspected when typical symptom of ataxia with concurrent is identified, it is sometimes difficult to diagnose hereditary ataxia without genetic test. Clinically, next generation sequencing technology has been developed and widely used for diagnosis of hereditary disease. Hereby, we experienced cases of genetically confirmed OPA1 mutation, which are presented with various clinical manifestations including ataxic gait and decreased visual acuity.
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Objective@#The International Cooperative Ataxia Rating Scale (ICARS) is a semiquantitative clinical scale for ataxia that is widely used in numerous countries. The purpose of this study was to investigate the validity and reliability of the Korean-translated version of the ICARS. @*Methods@#Eighty-eight patients who presented with cerebellar ataxia were enrolled. We investigated the construct validity using exploratory factor analysis (EFA) and confirmatory factor analysis (CFA). We also investigated the internal consistency using Cronbach’s α and intrarater and interrater reliability using intraclass correlation coefficients. @*Results@#The Korean-translated ICARS showed satisfactory construct validity using EFA and CFA. It also revealed good interrater and intrarater reliability and showed acceptable internal consistency. However, subscale 4 for assessing oculomotor disorder showed moderate internal consistency. @*Conclusion@#This is the first report to investigate the validity and reliability of the Korean-translated ICARS. Our results showed excellent construct and convergent validity. The reliability is also acceptable.
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Background@#Neurology in Korea is a field of clinical practice and research that has been developing for over 30 years, but due to its relatively short history, public awareness is low compared to other clinical fields. The Korean Neurological Association conducted a survey to evaluate the Korean public’s awareness of neurology and the necessity of a name change. This study details the second survey conducted on the topic, with the first being conducted in 2013. The two surveys were compared to identify how the public’s awareness of neurology has improved since 2013 and determine how this can be improved. @*Methods@#The survey was conducted by Gallup Korea, where 1,000 participants were randomly selected and interviewed through telephone surveys. @*Results@#Of the 1,000 participants, 78.5% were familiar with Neurology, similar to the results of the 2013 survey. Meanwhile, when asked about neurological diseases, only 27.9% of the participants recognized neurological diseases correctly. The most accurately recognized symptoms related to Neurology were tremors (57.8%), strokes (55.5%), and dizziness (49.7%). In 2013, 61.7% of the participants thought a name change was necessary. In comparison, only 38.4% responded it needed to change in the recent survey. @*Conclusions@#The preference for Neurology’s current name in Korea has improved since 2013 despite no significant change in the public’s awareness of neurology. More active public relations activities are needed to improve awareness of neurology in the future.
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Background@#Neurology in Korea is a field of clinical practice and research that has been developing for over 30 years, but due to its relatively short history, public awareness is low compared to other clinical fields. The Korean Neurological Association conducted a survey to evaluate the Korean public’s awareness of neurology and the necessity of a name change. This study details the second survey conducted on the topic, with the first being conducted in 2013. The two surveys were compared to identify how the public’s awareness of neurology has improved since 2013 and determine how this can be improved. @*Methods@#The survey was conducted by Gallup Korea, where 1,000 participants were randomly selected and interviewed through telephone surveys. @*Results@#Of the 1,000 participants, 78.5% were familiar with Neurology, similar to the results of the 2013 survey. Meanwhile, when asked about neurological diseases, only 27.9% of the participants recognized neurological diseases correctly. The most accurately recognized symptoms related to Neurology were tremors (57.8%), strokes (55.5%), and dizziness (49.7%). In 2013, 61.7% of the participants thought a name change was necessary. In comparison, only 38.4% responded it needed to change in the recent survey. @*Conclusions@#The preference for Neurology’s current name in Korea has improved since 2013 despite no significant change in the public’s awareness of neurology. More active public relations activities are needed to improve awareness of neurology in the future.
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Background@#and Purpose Screening tests for dementia such as the Mini Mental State Examination (MMSE) and the Montreal Cognitive Assessment are widely used, but there are drawbacks to their efficient use. There remains a need for a brief and easy method of assessing the activities of daily living (ADL) that can be administered to elderly individuals by healthcare workers. We have therefore developed a new scale named the Simple Observation Checklist for Activities of Daily Living (SOC-ADL). @*Methods@#We developed the SOC-ADL scale as a team of experts engaged in caring for individuals with dementia. This scale comprises eight items and was designed based on the Korean instrumental activities of daily living (K-IADL) scale and the Barthel activities of daily living scale (Barthel Index). The new scale was validated by enrolling 176 patients with cognitive dysfunction across 6 centers. Confirmatory factor analysis (CFA) and exploratory factor analysis (EFA) were performed. We assessed its concurrent validity by performing comparisons with the Korean-MMSE, Clinical Dementia Rating, Clinical Dementia Rating-Sum of Boxes, K-IADL, and Barthel Index, and its criterion validity by performing comparisons between mild cognitive impairment (MCI) and dementia. We also used Cronbach’s alpha to assess the interitem reliability. The appropriate cutoff values were determined by analyzing receiver operating characteristic curves, including the areas underneath them. @*Results@#EFA extracted one factor and CFA revealed that all of the model fits exceeded the minimum acceptable criteria. The SOC-ADL scores were strongly correlated with those of the other tools for dementia and could be used to differentiate MCI from dementia. Cronbach’s alpha values indicated that the results were reliable. The optimal cutoff value of the SOC-ADL for discriminating dementia from MCI was 3 points, which provided a sensitivity and specificity of 74.5% and 75.7%, respectively. @*Conclusions@#Our results demonstrate that the SOC-ADL is a valid and reliable tool for differentiating dementia from MCI based on an assessment of ADL. This new tool can be used for screening ADL in elderly subjects who have difficulty communicating, and to increase the efficiency of dementia screening at the population level.
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Background@#and Purpose: The Movement Disorder Society-Sponsored Revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) is widely used for estimating the symptoms of Parkinson’s disease. Translation and validation of the MDS-UPDRS is necessary for non-English speaking countries and regions. The aim of this study was to validate the Korean version of the MDS-UPDRS. @*Methods@#Altogether, 362 patients in 19 centers were recruited for this study. We translated the MDS-UPDRS to Korean using the translation-back translation method and cognitive pretesting. We performed both confirmatory and exploratory factor analyses to validate the scale.We calculated the comparative fit index (CFI) for confirmatory factor analysis, and used unweighted least squares for exploratory factor analysis. @*Results@#The CFI was higher than 0.90 for all parts of the scale. Exploratory factor analysis also showed that the Korean MDS-UPDRS has the same number of factors in each part as the English version. @*Conclusions@#The Korean MDS-UPDRS has the same overall structure as the English MDSUPDRS. Our translated scale can be designated as the official Korean MDS-UPDRS.
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@#Anti-aquaporin 4 antibody is the most common cause of neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oliogodendrocyte glycoprotein (MOG) antibody recently has emerged as another cause of NMOSD. Visual field defect can be observed as a manifestation of optic neuritis in patients with multiple sclerosis and NMOSD. However, homonymous hemianopia associated with a visual cortex lesion has been rarely reported in patients with multiple sclerosis and anti-aquaporin 4-positive NMOSD. Recently, we experienced a case of MOG-positive NMOSD who presented with a homonymous hemianopia associated with a visual cortex lesion
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BACKGROUND AND PURPOSE: There is accumulating evidence that epilepsy is caused by network dysfunction. We evaluated the hub reorganization of subcortical structures in patients with focal epilepsy using graph theoretical analysis based on diffusion-tensor imaging (DTI). In addition, we investigated differences in the values of diffusion tensors and scalars, fractional anisotropy (FA), and mean diffusivity (MD) of subcortical structures between patients with focal epilepsy and healthy subjects. METHODS: One hundred patients with focal epilepsy and normal magnetic resonance imaging (MRI) findings and 80 age- and sex-matched healthy subjects were recruited prospectively. All subjects underwent DTI to obtain data suitable for graph theoretical analysis. We investigated the differences in the node strength, cluster coefficient, eigenvector centrality, page-rank centrality measures, FA, and MD of subcortical structures between patients with epilepsy and healthy subjects. RESULTS: After performing multiple corrections, the cluster coefficient and the eigenvector centrality of the globus pallidus were higher in patients with epilepsy than in healthy subjects (p=0.006 and p=0.008, respectively). In addition, the strength and the page-rank centrality of the globus pallidus tended to be higher in patients with epilepsy than in healthy subjects (p=0.092 and p=0.032, respectively). The cluster coefficient of the putamen was lower in patients with epilepsy than in healthy subjects (p=0.004). The FA values of the caudate nucleus and thalamus were significantly lower in patients with epilepsy than in healthy subjects (p=0.009 and p=0.007, respectively), whereas the MD value of the thalamus was higher than that in healthy subjects (p=0.005). CONCLUSIONS: We discovered the presence of hub reorganization of subcortical structures in focal epilepsy patients with normal MRI findings, suggesting that subcortical structures play a pivotal role as a hub in the epilepsy network. These findings further reinforce the idea that epilepsy is a network disease.
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Humans , Anisotropy , Caudate Nucleus , Connectome , Diffusion , Epilepsies, Partial , Epilepsy , Globus Pallidus , Healthy Volunteers , Magnetic Resonance Imaging , Prospective Studies , Putamen , ThalamusABSTRACT
OBJECTIVE: Postural instability and gait disturbance are the cardinal symptoms associated with falling among patients with Parkinson’s disease (PD). The Tinetti mobility test (TMT) is a well-established measurement tool used to predict falls among elderly people. However, the TMT has not been established or widely used among PD patients in Korea. The purpose of this study was to evaluate the reliability and validity of the Korean version of the TMT for PD patients. METHODS: Twenty-four patients diagnosed with PD were enrolled in this study. For the interrater reliability test, thirteen clinicians scored the TMT after watching a video clip. We also used the test-retest method to determine intrarater reliability. For concurrent validation, the unified Parkinson’s disease rating scale, Hoehn and Yahr staging, Berg Balance Scale, Timed-Up and Go test, 10-m walk test, and gait analysis by three-dimensional motion capture were also used. We analyzed receiver operating characteristic curve to predict falling. RESULTS: The interrater reliability and intrarater reliability of the Korean Tinetti balance scale were 0.97 and 0.98, respectively. The interrater reliability and intra-rater reliability of the Korean Tinetti gait scale were 0.94 and 0.96, respectively. The Korean TMT scores were significantly correlated with the other clinical scales and three-dimensional motion capture. The cutoff values for predicting falling were 14 points (balance subscale) and 10 points (gait subscale). CONCLUSION: We found that the Korean version of the TMT showed excellent validity and reliability for gait and balance and had high sensitivity and specificity for predicting falls among patients with PD.
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Aged , Humans , Accidental Falls , Gait , Korea , Methods , Parkinson Disease , Reproducibility of Results , ROC Curve , Sensitivity and Specificity , Weights and MeasuresABSTRACT
With developing technology, quantitative analysis of gait and balance has been much interested in last decade. In gait analysis, three-dimensional motions capture and gait analysis using foot pressure is widely used. For measuring balance, posturography with force plate especially dynamic intervention has been developed. Recently wearable device containing accelerometer and gyro-sensor can assess long term monitoring for real living situation beyond lab based environment. Future studies of validity and reliability of quantitative analysis is necessary for clinical implication.
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Foot , Gait , Postural Balance , Reproducibility of ResultsABSTRACT
Optic perineuritis (OPN) is a rare form of orbital inflammatory disease of optic nerve sheath. The clinical presentation of OPN are known as distinct from demyelinating optic neuritis (ON). Recently, we have experienced a patient with bilateral idiopathic ON who had initial magnetic resonance imaging (MRI) feature of OPN. Serial MRI and funduscopic examination exhibited the bilateral ON findings. Serial MRI and funduscopic examination are needed in diagnosing OPN to differentiate to the temporal change of ON.
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Humans , Magnetic Resonance Imaging , Optic Nerve , Optic Neuritis , OrbitABSTRACT
No abstract available.
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Arteries , Choroid , Magnetic Resonance Angiography , VasculitisABSTRACT
No abstract available.
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Magnetic Resonance Imaging , Multiple Sclerosis , NeurosyphilisABSTRACT
Movement disorders are primarily associated with the basal ganglia and the thalamus; therefore, movement disorders are more frequently manifest after stroke compared with neurological injuries associated with other structures of the brain. Overall clinical features, such as types of movement disorder, the time of onset and prognosis, are similar with movement disorders after stroke in other structures. Dystonia and chorea are commonly occurring post-stroke movement disorders in basal ganglia circuit, and these disorders rarely present with tremor. Rarer movement disorders, including tic, restless leg syndrome, and blepharospasm, can also develop following a stroke. Although the precise mechanisms underlying the pathogenesis of these conditions have not been fully characterized, disruptions in the crosstalk between the inhibitory and excitatory circuits resulting from vascular insult are proposed to be the underlying cause. The GABA (gamma-aminobutyric acid)ergic and dopaminergic systems play key roles in post-stroke movement disorders. This review summarizes movement disorders induced by basal ganglia and thalamic stroke according to the anatomical regions in which they manifest.
Subject(s)
Basal Ganglia , Blepharospasm , Brain , Cerebrovascular Disorders , Chorea , Dystonia , gamma-Aminobutyric Acid , Movement Disorders , Prognosis , Restless Legs Syndrome , Stroke , Thalamus , Tics , TremorABSTRACT
BACKGROUND AND PURPOSE: We investigated the potential role of serum procalcitonin in differentiating tuberculosis meningitis from bacterial and viral meningitis, and in predicting the prognosis of tuberculosis meningitis. METHODS: This was a retrospective study of 26 patients with tuberculosis meningitis. In addition, 70 patients with bacterial meningitis and 49 patients with viral meningitis were included as the disease control groups for comparison. The serum procalcitonin level was measured in all patients at admission. Differences in demographic and laboratory data, including the procalcitonin level, were analyzed among the three groups. In addition, we analyzed the predictive factors for a prognosis of tuberculosis meningitis using the Glasgow Coma Scale (GCS) at discharge, and the correlation between the level of procalcitonin and the GCS score at discharge. RESULTS: Multiple logistic regression analysis showed that a low level of procalcitonin (≤1.27 ng/mL) independently distinguished tuberculosis meningitis from bacterial meningitis. The sensitivity and specificity for distinguishing tuberculosis meningitis from bacterial meningitis were 96.2% and 62.9%, respectively. However, the level of procalcitonin in patients with tuberculosis meningitis did not differ significantly from that in patients with viral meningitis. In patients with tuberculosis meningitis, a high level of procalcitonin (>0.4 ng/mL) was a predictor of a poor prognosis, and the level of procalcitonin was negatively correlated with the GCS score at discharge (r=-0.437, p=0.026). CONCLUSIONS: We found that serum procalcitonin is a useful marker for differentiating tuberculosis meningitis from bacterial meningitis and is also valuable for predicting the prognosis of tuberculosis meningitis.
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Humans , Bacteria , Glasgow Coma Scale , Logistic Models , Meningitis, Bacterial , Meningitis, Viral , Prognosis , Retrospective Studies , Sensitivity and Specificity , Tuberculosis , Tuberculosis, MeningealABSTRACT
Hashimoto's encephalopathy is a neurological disorder associated with high titers of antithyroid antibodies. The common neurologic symptoms are seizure, stroke-like episode, or mental change. However other atypical presentations, such as aphasia, myoclonus, ataxia, and cognitive dysfunction have also been described. We report hear a 44-year-old woman with Hashimoto's encephalopathy. The patient presented with ocular flutter as a principal manifestation, which is rare in Hashimoto's encephalopathy.
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Adult , Female , Humans , Antibodies , Aphasia , Ataxia , Myoclonus , Nervous System Diseases , Neurologic Manifestations , SeizuresABSTRACT
OBJECTIVES: Documented risk factors for obstructive sleep apnea include advanced age, male gender, hypertension, large neck circumference, and obesity; but some controversy remains regarding the risk factors, especially in Asians. In this study, we evaluated the risk factors for obstructive sleep apnea in snoring patients, and also analyzed the risk factors that could predict the severity of obstructive sleep apnea. METHODS: The inclusion criteria were patients 1) who visited our hospital with a chief complaint of snoring as witnessed by a sleep partner and 2) who underwent overnight polysomnography. The primary endpoint was the presence of obstructive sleep apnea as a dependent variable. RESULTS: One hundred forty-seven patients met the inclusion criteria. Of the 147 patients, 109 patients were diagnosed with obstructive sleep apnea. Multivariate analysis showed that old age and large neck circumference were significant independent variables for predicting the presence of obstructive sleep apnea, whereas hypertension and large neck circumference were independent variables for predicting the severity of obstructive sleep apnea. CONCLUSIONS: We demonstrated that neck circumference can be used to predict the presence as well as the severity of obstructive sleep apnea in snoring Asian patients.
Subject(s)
Humans , Male , Asian People , Hypertension , Multivariate Analysis , Neck , Obesity , Polysomnography , Risk Factors , Sleep Apnea Syndromes , Sleep Apnea, Obstructive , SnoringABSTRACT
BACKGROUND: Triphasic waves are one of the electroencephalographic patterns that can be usually seen in metabolic encephalopathy. The aim of this study is to compare the clinical and electrophysiologic profiles between patients with and without triphasic waves in metabolic encephalopathy, and reassess the significance of triphasic waves in metabolic encephalopathy. METHODS: We recruited 127 patients with metabolic encephalopathy, who were admitted to our hospital. We divided these admitted patients into two groups; those with and without triphasic waves. We analyzed the difference of duration of hospitalization, mortality rate during admission, Glasgow Coma Scale, severity of electroencephalographic alteration, and presence of acute symptomatic seizures between these two groups. RESULTS: Of the 127 patients with metabolic encephalopathy, we excluded 67 patients who did not have EEG, and 60 patients finally met the inclusion criteria for this study. Patients with triphasic waves had more severe electroencephalographic alterations, lower Glasgow Coma Scale, and more acute symptomatic seizures than those without triphasic waves. After adjusting the clinical variables, Glasgow Coma Scale and acute symptomatic seizures were only significantly different between patients with and without triphasic waves. CONCLUSIONS: We demonstrated that patients with triphasic waves in metabolic encephalopathy had more significant impairment of the brain function.