Validity and Reliability of the Korean-Translated Version of the International Cooperative Ataxia Rating Scale in Cerebellar Ataxia

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.

Validation Study of the Official Korean Version of the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale

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.

Validation of the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale

Background@#and PurposeImpulse-control disorder is an important nonmotor symptom of Parkinson's disease (PD) that can lead to financial and social problems, and be related to a poor quality of life. A nationwide multicenter prospective study was performed with the aim of validating the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale (K-QUIP-RS). @*Methods@#The K-QUIP-RS was constructed using forward and backward translation, and pretesting of the prefinal version. PD patients on stable medical condition were recruited from 27 movement-disorder clinics. Participants were assessed using the K-QUIP-RS and evaluated for parkinsonian motor and nonmotor statuses and for PD-related quality of life using a predefined evaluation battery. The test–retest reliability of the K-QUIP-RS was assessed over an interval of 10–14 days, and correlations between the KQUIP-RS and other clinical scales were analyzed. @*Results@#This study enrolled 136 patients. The internal consistency of the K-QUIP-RS was indicated by a Cronbach's α coefficient of 0.846, as was the test–retest reliability by a Guttman split-half coefficient of 0.808. The total K-QUIP-RS score was positively correlated with the scores for depression and motivation items on the Unified PD Rating Scale (UPDRS), Montgomery-Asberg Depression Scale, and Rapid-Eye-Movement Sleep-Behavior-Disorders Questionnaire. The total K-QUIP-RS score was also correlated with the scores on part II of the UPDRS and the PD Quality of Life-39 questionnaire, and the dopaminergic medication dose. @*Conclusions@#The K-QUIP-RS appears to be a reliable assessment tool for impulse-control and related behavioral disturbances in the Korean PD population.

Subtypes of Sleep Disturbance in Parkinson's Disease Based on the Cross-Culturally Validated Korean Version of Parkinson's Disease Sleep Scale-2

Validation of the Korean Version of the Scales for Outcomes in Parkinson's Disease-Sleep

BACKGROUND: Sleep problems commonly occur in patients with Parkinson's disease (PD), and are associated with a lower quality of life. The aim of the current study was to translate the English version of the Scales for Outcomes in Parkinson's Disease-Sleep (SCOPA-S) into the Korean version of SCOPA-S (K-SCOPA-S), and to evaluate its reliability and validity for use by Korean-speaking patients with PD. METHODS: In total, 136 patients with PD from 27 movement disorder centres of university-affiliated hospitals in Korea were enrolled in this study. They were assessed using SCOPA, Hoehn and Yahr Scale (HYS), Unified Parkinson's Disease Rating Scale (UPDRS), Parkinson's Disease Sleep Scale 2nd version (PDSS-2), Non-motor Symptoms Scale (NMSS), Montgomery Asberg Depression Scale (MADS), 39-item Parkinson's Disease Questionnaire (PDQ39), Neurogenic Orthostatic Hypotension Questionnaire (NOHQ), and Rapid Eye Movement Sleep Behaviour Disorder Questionnaire (RBDQ). The test-retest reliability was assessed over a time interval of 10–14 days. RESULTS: The internal consistency (Cronbach's α-coefficients) of K-SCOPA-S was 0.88 for nighttime sleep (NS) and 0.75 for daytime sleepiness (DS). Test-retest reliability was 0.88 and 0.85 for the NS and DS, respectively. There was a moderate correlation between the NS sub-score and PDSS-2 total score. The NS and DS sub-scores of K-SCOPA-S were correlated with motor scale such as HYS, and non-motor scales such as UPDRS I, UPDRS II, MADS, NMSS, PDQ39, and NOHQ while the DS sub-score was with RBDQ. CONCLUSION: The K-SCOPA-S exhibited good reliability and validity for the assessment of sleep problems in the Korean patients with PD.

Erratum to: The KMDS-NATION Study: Korean Movement Disorders Society Multicenter Assessment of Non-Motor Symptoms and Quality of Life in Parkinson's Disease NATION Study Group

The original version of this article contained wrong informations of some authors which should be changed.

Patients and Their Caregivers' Burdens for Parkinson's Disease in Korea

OBJECTIVE: Many patients with Parkinson’s disease (PD) suffer from motor and non-motor symptoms. According to these variable symptoms of PD, patients or caregivers have a poorer quality of life than patients with other neurodegenerative diseases. Since the difficulties are varied for all patients, prioritizing their difficulties differs among all cases. The goal of this study was to investigate the burdens of PD among the caregivers as well as patients and to identify areas requiring aid from the government. METHODS: We surveyed the awareness and perceptions of PD in patients and caregivers of PD by a face-to-face questionnaire. The questionnaire was divided into three sections: symptoms of PD (part A), desire for policies (part B), and difficulties faced by their caregivers (part C). Part A comprised 8 questions, Part B had 2 questions, and Part C had 3 questions. RESULTS: In total, 853 subjects (702 patients and 151 caregivers) were enrolled in this study. The major difficulties experienced by PD patients were physical (67%), psychiatric (60%) and socio-economic (52%). Assessing the physical difficulties, more than half the patients experienced severe difficulties (29% very severe, 39% severe). Psychiatric difficulties were assessed as severe (35%) and very severe (21%) among the patients. Severe difficulties were also experienced socio-economically, at 52% in patients and 49% in caregivers, especially among patients in their fifties (58%) and those with their spouse (65%) as caregivers. The topmost need was the introduction of new technology for treatment of PD (62%), followed by relief of costs for treatment (38%) and a family support system (31%). The majority (91%) of the patients were diagnosed with PD within two years after onset of symptoms. CONCLUSION: We know that the difficulties of PD and the needs for government assistance are different between patients and caregivers. These results emphasize that perceiving the difficulties and needs of patients and caregivers early can help to prevent and ameliorate the burden of disease.

Validation of the Korean Version of the Scale for Outcomes in Parkinson's Disease-Autonomic

OBJECTIVE: Autonomic symptoms are commonly observed in patients with Parkinson's disease (PD) and often limit the activities of daily living. The Scale for Outcomes in Parkinson's disease-Autonomic (SCOPA-AUT) was developed to evaluate and quantify autonomic symptoms in PD. The goal of this study was to translate the original SCOPA-AUT, which was written in English, into Korean and to evaluate its reliability and validity for Korean PD patients. METHODS: For the translation, the following processes were performed: forward translation, backward translation, expert review, pretest of the pre-final version and development of the final Korean version of SCOPA-AUT (K-SCOPA-AUT). In total, 127 patients with PD from 31 movement disorder clinics of university-affiliated hospitals in Korea were enrolled in this study. All patients were assessed using the K-SCOPA-AUT and other motor, non-motor, and quality of life scores. Test-retest reliability for the K-SCOPA-AUT was assessed over a time interval of 10−14 days. RESULTS: The internal consistency and reliability of the K-SCOPA-AUT was 0.727 as measured by the mean Cronbach's α-coefficient. The test-retest correlation reliability was 0.859 by the Guttman split-half coefficient. The total K-SCOPA-AUT score showed a positive correlation with other non-motor symptoms [the Korean version of non-motor symptom scale (K-NMSS)], activities of daily living (Unified Parkinson's Disease Rating Scale part II) and quality of life [the Korean version of Parkinson's Disease Quality of Life 39 (K-PDQ39)]. CONCLUSION: The K-SCOPA-AUT had good reliability and validity for the assessment of autonomic dysfunction in Korean PD patients. Autonomic symptom severities were associated with many other motor and non-motor impairments and influenced quality of life.

Nonalcoholic Wernicke's Encephalopathy With Diffuse Cortical Involvement

Wernicke's encephalopathy is an acute neurological deterioration due to a reversible brain lesion caused by thiamine deficiency. Most of the affected patients are thiamine-depleted alcoholics, and the condition usually involves the medial thalami, mammillary bodies, and periaqueductal area. However, there are rare reports of lesions in the cerebellum and cerebral cortex, especially in patients with nonalcoholic Wernicke's encephalopathy. We report a case of nonalcoholic Wernicke's encephalopathy involving atypical diffuse cortical regions, and review previously reported cases.

Progressive Supranuclear Palsy-Like Syndrome after Surgical Repair of Chronic Ascending Aorta Dissection

A 70-year-old woman underwent cardiopulmonary bypass surgery for aorta dissection. After 10 days she developed a vertical gaze palsy, and 2 months later she presented with dysarthria, bradykinesia, postural instability, blepharospasm, and truncal tilt to the left. Brain imaging indicated old lacunes in the bilateral thalamus. Her symptoms remained unchanged during a 4-year follow-up, which seems to be incompatible with progressive supranuclear palsy (PSP). However, the clinical features of this case were suggestive of PSP-like syndrome after cardiopulmonary bypass surgery.

The KMDS-NATION Study: Korean Movement Disorders Society Multicenter Assessment of Non-Motor Symptoms and Quality of Life in Parkinson's Disease NATION Study Group

BACKGROUND AND PURPOSE: Nonmotor symptoms (NMS) in Parkinson's disease (PD) have multisystem origins with heterogeneous manifestations that develop throughout the course of PD. NMS are increasingly recognized as having a significant impact on the health-related quality of life (HrQoL). We aimed to determine the NMS presentation according to PD status, and the associations of NMS with other clinical variables and the HrQoL of Korean PD patients. METHODS: We surveyed patients in 37 movement-disorders clinics throughout Korea. In total, 323 PD patients were recruited for assessment of disease severity and duration, NMS, HrQoL, and other clinical variables including demographics, cognition, sleep scale, fatigability, and symptoms. RESULTS: In total, 98.1% of enrolled PD subjects suffered from various kinds of NMS. The prevalence of NMS and scores in each NMS domain were significantly higher in the PD group, and the NMS worsened as the disease progressed. Among clinical variables, disease duration and depressive mood showed significant correlations with all NMS domains (p<0.001). NMS status impacted HrQoL in PD (rS=0.329, p<0.01), and the association patterns differed with the disease stage. CONCLUSIONS: The results of our survey suggest that NMS in PD are not simply isolated symptoms of degenerative disease, but rather exert significant influences throughout the disease course. A novel clinical approach focused on NMS to develop tailored management strategies is warranted to improve the HrQoL in PD patients.

Relationship between Changes in Fatigue and Exercise by Follow-Up Period / 가정의학회지

BACKGROUND: Fatigue is one of the most common presenting symptoms in primary care in Korea. In this study, we aimed to determine the effect of exercise intervention on the severity of fatigue of unknown medical cause during a period of follow-up. METHODS: We used the data collected from an outpatient fatigue clinic in Seoul National University Bundang Hospital. The study was conducted from March 3, 2010 to May 31, 2014. We measured the body mass index of each patient and evaluated variables including lifestyle factors (smoking, alcohol consumption, and regular exercise), quality of sleep, anxiety, depression, stress severity, and fatigue severity using questionnaires. A total of 152 participants who completed questionnaires to determine changes in fatigue severity and the effect of exercise for each period were evaluated. We used univariate analysis to verify possible factors related to fatigue and then conducted multivariate analysis using these factors and the literature. RESULTS: Of 130 patients with the complaint of chronic fatigue for over 6 months, over 90 percent reported moderate or severe fatigue on the Fatigue Severity Scale and Brief Fatigue Inventory questionnaires. The fatigue severity scores decreased and fatigue improved over time. The amount of exercise was increased in the first month, but decreased afterwards. CONCLUSION: There was no significant relationship between changes in the amount of exercise and fatigue severity in each follow-up period. Randomized controlled trials and a cohort study with a more detailed exercise protocol in an outpatient setting are needed in the future.

The Effect of Omega-3 Fatty Acid Supplementation on Cerebral Blood Flow and Vascular Resistance: A Preliminary Study / 대한임상신경생리학회지

BACKGROUND: The effects of omega-3 polyunsaturated fatty acids (PUFAs) on cerebral vessels have not been clarified until now. Thus we investigated the efficacy of omega-3 PUFAs supplementation on cerebral blood flow velocity and vascular resistance via transcranial doppler (TCD). METHODS: Consecutive twenty patients (13 male and 7 female) with at least 1 cerebrovascular risk factor or a known cerebrovascular disease were enrolled. Patients were treated with omega-3 PUFAs (1 g, two times per day) for 12 weeks. Cerebral blood flow velocity, resistance index, and pulsatile index were checked before and after 12 weeks of treatment using TCD. RESULTS: The change of resistance index in right MCA (from 0.58 +/- 0.07 to 0.55 +/- 0.07, p = 0.042) and left PCA (from 0.56 +/- 0.07 to 0.53 +/- 0.06, p = 0.037) showed significant improvement after 12 weeks of omega-3 PUFAs treatment. The changes in other vessels, however, failed to show any significant changes compared to the baseline. CONCLUSIONS: Omega-3 PUFAs treatment showed feasible efficacies for cerebral vascular resistances in this open label trial. To confirm these results, larger samples of patients and longer period of follow-up is warranted.

Barrier Factors to the Completion of Diabetes Education in Korean Diabetic Adult Patients: Korea National Health and Nutrition Examination Surveys 2007-2012 / 가정의학회지

BACKGROUND: Diabetes is a disease with high social burdens and is expected to increase gradually. A long-term management is essential for the treatment of diabetes, requiring patient self-cares. Diabetes education is important for such self-cares, but it does not sufficiently take place. In addition, little studies have been conducted on the barriers to the completion of diabetes education. This study, thus, aimed to analyze the factors related to the completion of diabetes education and investigate its barriers. METHODS: Of 50,405 respondents to the fourth and fifth Korea National Health and Nutrition Examination Survey, a total of 3,820 were selected for the analysis, excluding those aged 29 or younger and those with missing values. The completion of diabetes education was set as a dependent variable and an analysis was made on the factors that affect the dependent variable. A multivariable logistic regression was employed for the analysis. RESULTS: Lower educational level was associated with less diabetes education, and the degree of diabetes education was lower in the group with male, the group that didn't have a family history or was not aware of a family history, the group that was not currently aware of diabetes and the group without a spouse. There was no difference in the completion of diabetes education by underlying diseases, family income level, age, residing area, economic activity status, insurance coverage, smoking, and drinking. CONCLUSION: Diabetes education is of importance for the treatment and management of diabetes. Currently, however, diabetes education is not sufficiently carried out in Korea. The completion rate of diabetes education was low in male, patients without or not knowing a family history, patients who were not currently aware of their diabetes, patients without a spouse, and patients with low educational level. Therefore, encouraging these patients to take the education will be a more effective approach to increase the completion rate of diabetes education.

Associations between Metabolic Syndrome and Inadequate Sleep Duration and Skipping Breakfast / 가정의학회지

BACKGROUND: The prevalence of metabolic syndrome is increasing worldwide, and previous studies have shown that inadequate sleep duration and skipping breakfast may be related to metabolic syndrome. Therefore, we investigated the effects of inadequate sleep and skipping breakfast on metabolic syndrome using data from the Korea National Health and Nutrition Examination Survey (KNHANES) IV & V reports (2007-2009 and 2010-2012, respectively). METHODS: The sample included 12,999 subjects who participated in the KNHANES IV & V. Sleep duration and breakfast eating were self-reported, and metabolic syndrome was defined according to the modified National Cholesterol Education Program Adult Treatment Panel III guidelines. Subjects were divided into 12 groups according to breakfast eating and sleep duration patterns, and multiple logistic regression analyses adjusted for age, sex, household income, education level, smoking status, alcohol drinking, physical activity, and total daily energy intake were conducted. RESULTS: In subjects under 50 years of age, sleeping less than 6 hours was significantly associated with increased metabolic syndrome except among those who ate breakfast on only 1 of the past 2 days. In subjects over 50 years of age, sleeping less than 6 hours was significantly associated with a decreased risk of metabolic syndrome among those who ate breakfast on both days. CONCLUSION: In conclusion, significant associations between metabolic syndrome and sleep duration were identified, and these associations differed according to age group.

A Case of Mammary Analogue Secretory Carcinoma Arising from Parotid Gland / 대한이비인후과학회지

Mammary analogue secretory carcinoma is a recently described salivary gland tumor. Histopathologically, mammary analogue secretory carcinoma resembles both acinic cell carcinoma and secretory breast carcinoma but also exhibit some differences. We present a case of mammary analogue secretory carcinoma of the parotid gland to help further characterize this rare tumor.

A novel mutation of CACNA1A gene in episodic ataxia type 2 family in Korea

Episodic ataxia type 2 (EA-2) is a rare disorder presenting with paroxysmal vertigo and cerebellar dysfunction. EA-2 is known to be caused by mutations of the CACNA1A gene on chromosome 19q13. We examined a family of EA-2 with a novel mutation of the CACNA1A gene showing characteristic ocular symptoms. A-36-year woman visited our hospital with paroxysmal vertigo. When she experienced vertigo attack, she also suffered from gait disturbance, dysarthria, and ataxia. She complained that she could not ride in a car or a train that moved fast, because she could not visually follow the moving objects. Her mother, grandmother, and uncle also complained of similar symptoms. Video nystagmographic findings showed loss of optokinetic nystagmus. We found a novel missense mutation, R279C (c.835C>T), on exon 6 in the CACNAIA gene. This is the first report of a family with new mutation of EA-2 in Korea.

Hearing Injury Evaluation: Current Status and Medicolegal Considerations / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: In disability rating of hearing injury, there are some medicolegal considerations, such as discrepancy in estimating values between McBride system and the American Medical Association system, and difficulties in judging the hearing level in boundary cases. We have therefore felt the need of complementary hearing disability evaluation methods that reflect reality. As a first step, we have pointed out current situation and problems in legal advisory cases of hearing injury. SUBJECTS AND METHOD: For the past 14 years, we have provided legal advisory on hearing injury for 121 cases for courts (group A) or insurance companies (group B). Eleven cases of 'aggravation of disability' were excluded. Data were summarized and tested statistically. We have used unpaired t-test for continuous variables such as age, hearing, hearing disability, disability rating, Fisher's exact test for dichotomous variable such as gender, group (A/B), and Pearson's correlation test for correlation coefficients between continuous variables. RESULTS: Subjects were more frequently males (3:1) and in the fifth decade of life. The averages of audiometry were around 40 dB. There were significant differences in hearing and disability rating between younger and older group. Group B is significantly older than group A. The type of disability or accident did not influence hearing and disability rating. CONCLUSION: This paper could help figure out the current situation with respect to medicolegal considerations of hearing injury discrepancies in disability rating and could also serve well to recognize the necessity for a complementary hearing disability evaluation method, especially for borderline hearing level.

Behavioral and psychological symptoms in Korean patients with mild Alzheimer’s disease: Preliminary study

Behavioral and psychological symptoms of dementia are major components of Alzheimer’s disease. In this study, we aimed at investigating the prevalence and severity of behavioral and psychological symptoms of dementia in very mild to mild Alzheimer’s disease. Forty-four patients with Alzheimer’s disease who visited the neurology outpatient clinic of Seoul Medical Center were included. A trained neurologist or a supervised test technician administered the neuropsychological test, the Seoul Neuropsychological Screening Battery including Mini Mental State Examination and Clinical Dementia Rating. The instrument used for assessing behavioral and psychological symptoms of dementia was Neuropsychiatric Inventory-Questionnaire. In order of prevalence, apathy, depression, irritability, anxiety, and agitation were the most common symptoms occurring in very mild-to-mild Alzheimer’s disease group. The prevalence and severity of behavioral and psychological symptoms of dementia were found to be in positive correlation with the Clinical Dementia Rating scores. Behavioral and psychological symptoms of dementia are found even in the very early stage of Alzheimer’s disease, apathy and depression being the most common symptoms. Physicians should be aware of this when managing dementia patients.

A Survey of the Use of Antiepileptic Drugs in Stroke Patients

BACKGROUND: Seizures occur in 2-20% of stroke patients. Recent studies have reported that post-stroke seizures are associated with poorer functional outcomesand higher mortality. However there are no official guidelines on how to use antiepileptic drugs (AEDs) in stroke-related seizures. In this study we surveyed neurologists and neurosurgeons and compared the responses of subgroups categorized by department, specialty and workplace discrimination using a questionnaire containing questions concerning the present tendency to use AEDs in stroke patients. METHODS: 256 neurologists and neurosurgeons participated in the survey. The research instrument was a questionnaire comprising 9 parts and 30 questions. The questions concerned stroke mechanism, the prophylactic use of AEDs, and the choice of AED in early and late onset post-stroke seizures. RESULTS: Tendencies to use prophylactic AEDs in stroke differed depending on specialty and workplace(neurologist vs. neurosurgeon; 17.8% vs. 83.1%, p<0.001, hospital vs. university staff; 46.2% vs. 28.4%, p=0.05). The most commonly used prophylactic AEDs were valproic acid (75%) and levetiracetam (60%). Carbamazepine was the most commonly used AED and phenytoin and phenobarbital were still used in all subgroups to treat post-stroke seizures. CONCLUSIONS: There are significant differences between neurologists (17.8%) and neurosurgeons (83.1%) in the use of prophylactic AEDs after stroke. Valproic acid and levetiracetam are considered first-line prophylactic AEDs by neurosurgeon. Phenytoin and phenobarbital are still used in post-stroke seizure although they have been reported to have an adverse influence on motor recovery. We suggest that proper guidelines should be established for the use of AEDs in stroke-related seizures.