No abstract available.
Central Nervous System* ; Myopathies, Nemaline*

No abstract available.
Hypokalemia* ; Leukoencephalopathies*

No abstract available.
Humans ; Hydrocephalus* ; Parkinsonian Disorders*

No abstract available.
Myoclonus*

BACKGROUND AND PURPOSE: Responses to repetitive nerve stimulation (RNS) in patients with muscle-specific tyrosine kinase (MuSK) antibody (Ab)-positive myasthenia gravis (MG) vary depending on the muscles tested. We analyzed the RNS responses of limb and facial muscles in MuSK-Ab-positive and acetylcholine receptor (AChR)-Ab-negative MG (MuSK MG) and MuSK-Ab-negative and AChR-Ab-negative [double-seronegative (DSN)] MG patients. METHODS: We retrospectively compared RNS responses between 45 MuSK MG and 29 DSN MG. RNS was applied to the abductor digiti minimi, flexor carpi ulnaris, trapezius, orbicularis oculi, and nasalis muscles. RESULTS: Abnormal RNS responses in limb muscles were observed in 22.2 and 58.6% of MuSK MG and DSN MG patients, respectively, with abnormal facial responses observed in 77.8 and 65.5%, and abnormal responses observed in any of the five muscles in 86.7 and 72.4%. Abnormal RNS responses in the abductor digiti minimi or flexor carpi ulnaris were less frequent in MuSK MG (8.9 and 15.6%, respectively) than in DSN MG (37.9 and 55.2%), whereas the findings for other muscles were not significantly different between the groups. Abnormal facial responses but normal limb responses were independently associated with MuSK MG (odds ratio=5.224, 95% confidence interval=1.300–20.990). CONCLUSIONS: Abnormal RNS responses primarily in facial muscles without involvement of limb muscles were more pronounced in MuSK MG than in DSN MG. RNS of both facial and limb muscles in AChR-Ab-negative MG can increase the test sensitivity and aid in early suspicion of MuSK MG.
Acetylcholine ; Extremities ; Facial Muscles ; Humans ; Muscles ; Myasthenia Gravis* ; Protein-Tyrosine Kinases ; Retrospective Studies ; Superficial Back Muscles

BACKGROUND AND PURPOSE: There are few studies that have investigated predictive factors related to migraine prophylaxis of which produced inconsistent results. The aim of this study was to identify factors that can predict the treatment response to topiramate prophylaxis in pediatric patients with migraine. METHODS: One hundred and thirteen patients who were older than 7 years and received topiramate for at least 3 months were recruited from the Seoul National University Bundang Hospital outpatient clinic from 2005 to 2014. A positive response was defined as a reduction of more than 50% in the number of migraine episodes after topiramate treatment. Proposed predictive factors such as migraine characteristics including severity and frequency were assessed, as were other data on sex, disease duration, associated symptoms, family history, and impairment of daily activities. RESULTS: Seventy patients (61.9%) responded to prophylactic treatment with topiramate. Patients who experienced significant impairment in daily activities showed significant benefit from the treatment (p=0.004). Sex, the severity, frequency, and duration of migraine episodes, disease duration, treatment duration, age at onset, and associated symptoms were not significantly related to a response to topiramate treatment. CONCLUSIONS: Migraine characteristics and associated symptoms were not significantly related to a response to topiramate treatment. However, patients with significant impairment in daily activities showed significant benefit from the treatment, and so prophylactic topiramate treatment should be strongly encouraged in this patient group.
Age of Onset ; Humans ; Migraine Disorders* ; Outpatient Clinics, Hospital ; Seoul

BACKGROUND AND PURPOSE: The quality of anticoagulation is critical for ensuring the benefit of warfarin, but this has been less well studied in Korean ischemic stroke patients with atrial fibrillation (AF). METHODS: This study retrospectively analyzed the data of patients who had an AF-related ischemic stroke and were treated with long-term warfarin therapy in 16 Korean centers. The quality of warfarin therapy was primarily assessed by the time in therapeutic range [TTR; international normalized ratio (INR), 2.0–3.0] and additionally by the proportion of INR values within the therapeutic range. RESULTS: The long-term warfarin-treated cohort comprised 1,230 patients. They were aged 70.1±9.7 years (mean±SD), 42.5% were female, and their CHA₂DS₂-VASc score was 4.75±1.41. The TTR analysis included 33,941 INR measurements for 27,487 months: per patients, 27.6 (SD, 22.4) INR measurements for 22.4 (SD, 12.9) months. The mean TTR of individual patients was 49.1% (95% confidence interval, 47.9–50.3%), and the TTR quartiles were <34.5, 34.5–49.1, 49.1–64.5%, and >64.5%. None of the 16 centers achieved a mean TTR of >60%. Of all INR measurements, 44.6% were within the therapeutic range, 41.7% were <2.0, and 13.7% were >3.0. CONCLUSIONS: In Korean ischemic stroke patients who had AF, the quality of warfarin therapy was low and might be inadequate to effectively prevent recurrent stroke or systemic embolism.
Atrial Fibrillation* ; Cohort Studies ; Embolism ; Female ; Humans ; International Normalized Ratio ; Observational Study* ; Retrospective Studies* ; Stroke* ; Warfarin*

BACKGROUND AND PURPOSE: There has been no nationwide population-based study of the prevalence of self-perceived snoring/apnea in Korean adolescents. The purpose of this study was to estimate prevalence of self-perceived snoring/apnea in Korean high-school students and to evaluate their association with daytime sleepiness. METHODS: An online survey was used to investigate 12,672 students at 75 high schools across the 15 nationwide districts of South Korea. The variables were obtained using a self-reported questionnaire. The students answered questions about self-perceived snoring/apnea during the past 30 days. Daytime sleepiness was measured using a validated Korean version of the Epworth Sleepiness Scale, which was modified for adolescents. Covariates were the sex, school grade, frequency of self-perceived snoring/apnea, body mass index, sleep duration during a school day, and subjective perception of sleep duration. RESULTS: The prevalence of self-perceived snoring/apnea was 22.8% (26.4% for males vs. 18.8% for females, p<0.001) and 9.2% (10.5% for males vs. 7.7% for females, p<0.001). Obesity was significantly associated with self-perceived snoring [odds ratio (OR)=2.18, 95% confidence interval (CI)=1.94–2.46] and apnea (OR=1.57, 95% CI=1.33–1.86). Multivariate analysis showed that any frequency of self-perceived snoring/apnea was significantly associated with excessive daytime sleepiness (EDS). The OR of EDS increased significantly with the frequency of snoring and apnea. Female, sleep duration of <5 hours during a school day, and subjective perceptions of insufficient and considerable sleep durations were also significantly associated with EDS. CONCLUSIONS: The prevalence of self-perceived snoring/apnea was significantly higher in students who were male and obese. Students with self-perceived snoring/apnea exhibited more significant EDS and an increased risk of EDS as the frequency of snoring and apnea increased.
Adolescent ; Apnea* ; Body Mass Index ; Female ; Humans ; Korea ; Male ; Multivariate Analysis ; Obesity ; Prevalence* ; Snoring*

BACKGROUND AND PURPOSE: We aimed to determine the correlation between subjective evaluations of mood and cognitive functions by patients and informants, and the findings of a battery of neuropsychological tests. METHODS: We analyzed 74 subjects recruited from a general neurology clinic, comprising 37 patients with cognitive complaints and 37 informants (either relatives or caregivers in close contact with the patients). Four ordinal scales concerning recent memory, verbal expression, initiative, and mood were correlated with the findings of a series of neuropsychological tests and questionnaires using the tau b coefficient. RESULTS: The scores for the patients on the scales were most strongly correlated with scores on the 15-item Geriatric Depression Scale (GDS-15), while the scores for the informants were most strongly correlated with scores on GDS-15, the Informant Questionnaire on Cognitive Decline, and the Functional Activities Questionnaire (FAQ). The most significant correlation was between the initiative scale from informants and FAQ (tau b=-0.591, p<0.001), and it was the only one that remained significant after correcting for multiple testing (p Holm=0.013). CONCLUSIONS: Cognitive complaints from patients mainly reflect their mood, whilst informant reports mainly reflect both the functional ability and mood of the patients.
Caregivers ; Cognition* ; Dementia ; Depression ; Humans ; Memory ; Neurology* ; Neuropsychological Tests ; Weights and Measures

BACKGROUND AND PURPOSE: To determine the risk of Parkinson's disease (PD) in relation to erectile dysfunction (ED) based on the National Health Insurance Research Database in Taiwan. METHODS: We identified 3,153 patients who were newly diagnosed with ED between January 1, 2004 and December 31, 2010. A total of 12,612 randomly selected people without ED served as healthy controls. All of the study subjects were followed-up from the index date to the date of PD diagnosis, withdrawal from the National Health Insurance program, or the end of 2012 whichever occurred first. RESULTS: The incidence density rate of PD was 1.52-fold higher in the ED cohort than the non-ED cohort (3.44 vs. 1.64 per 1,000 person-years), with an adjusted hazard ratio (HR) of 1.52 [95% confidence interval (CI)=1.09–2.12]. The combined effects on patients with ED and diabetes as well as hypertension showed a significant combined association with the PD risk compared with patients without ED, counterpart comorbidities, or medication use. The adjusted HR of PD for ED was higher for diabetes (2.82, 95% CI=1.42–5.63) and hypertension (2.19, 95% CI = 1.35–3.55). CONCLUSIONS: ED leads to an increased risk of PD. ED patients with diabetes or hypertension have an elevated risk of PD.
Cohort Studies ; Comorbidity* ; Diagnosis ; Erectile Dysfunction* ; Humans ; Hypertension ; Incidence ; Longitudinal Studies* ; Male ; National Health Programs ; Parkinson Disease* ; Taiwan