Real-world prescribing trends of valproate in women with epilepsy in Korea.

Avoiding valproate is recommended in women of childbearing age due to possible teratogenicity and infertility. We aimed to examine the recent trend of valproate prescriptions in Korea to review the adequacy of anticonvulsant prescriptions in women with epilepsy (WWE). Oral valproate utilization was assessed using nationwide and unselected data from the Korean National Health Insurance Service from 2009 to 2017. The temporal trends of the proportions of valproate prescriptions were analyzed using the Poisson regression model and expressed as average annual percentage change (AAPC). Among the WWE of childbearing age, valproate was prescribed in 37.0% overall and 29.4% as initial prescription in 2017. The proportion of valproate utilization showed a decreasing trend in overall prescription (AAPC = -1.10%) and initial prescription (AAPC = -2.63%). However, the proportion was static over time in the initial monotherapy group (AAPC = -0. 53%), while it was significantly decreasing in the initial polytherapy group (AAPC = -8.25%). A noticeable proportion of WWE was still being prescribed valproate in Korea. In particular, the use of valproate for initial monotherapy has not decreased over the past nine years. This result calls not only for reinforcement of education regarding anticonvulsant selection but also for monitoring the actual prescription.

Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

Comparative Study of the Effect of 5,6-Dihydroergosterol and 3-epi-5,6-dihydroergosterol on Chemokine Expression in Human Keratinocytes.

5,6-Dihydroergosterol-glucose is an organic synthetic derivative of spinasterol-glucose, which has potent anti-inflammatory activity. We previously synthesized alpha and beta anomers of DHE-glycosides and compared their inhibitory activity on CCL17 and CCL22 mRNA expression induced by TNF-α/IFN-γ in activated HaCaTs. Recently, we synthesized a new type of DHE-glycosides, 3-epi-5,6-dihydroergosterol(3-epi-DHE)-glycosides, and compared its inhibitory activity on mRNA expression levels of CCL17 and CCL22 in TNF-α/IFN-γ-induced HaCaT. DHE-Xly did not affect TNF-α/IFN-γ induced CCL17 and CCL22 mRNA expression in HaCaTs, however, 3-epi-DHE-Xly strongly inhibited TNF-α/IFN-γ induced CCL17 and CCL22 mRNA expression levels in human keratinocytes. These results provide important clues for development of chronic dermatitis treatment via inhibition of chemokine expression using DHE derivatives.

Effect of Group-Task-Oriented Training on Gross and Fine Motor Function, and Activities of Daily Living in Children with Spastic Cerebral Palsy.

Aims: To determine the effects of group-task-oriented training (group-TOT) on gross and fine motor function, activities of daily living (ADL) and social function of children with spastic cerebral palsy (CP).Methods: Eighteen children with spastic CP (4-7.5 years, gross motor function classification system level I-III) were randomly assigned to the Group-TOT (9 children received group-TOT for 1 hour, twice a week for 8 weeks) or the comparison group (9 children received individualized traditional physical and occupational therapy). The Gross Motor Function Measure (GMFM)-88, the Bruininks-Oseretsky Test of Motor Proficiency 2nd edition (BOT-2), and the Pediatric Evaluation of Disability Inventory (PEDI) were administered before and after the intervention, and in the Group-TOT, 16 weeks after the intervention.Results: Children in the Group-TOT showed significant improvements in the GMFM-88 standing and walking/running/jumping subscales, the BOT-2 manual dexterity subscale, and the PEDI social function subscale (p < 0.05); changes were maintained 16 weeks after the intervention ended. In contrast, the comparison group improved in only the BOT-2 fine motor integration subscale (p < 0.05).Conclusions: The findings provide evidence of effectiveness of group-TOT in improving gross and fine motor function, and social function in children with CP.

The prevalence and clinical significance of sleep disorders in acute ischemic stroke patients-a questionnaire study.

PURPOSE: Sleep disturbances are frequently reported in stroke patients and associated with the outcome of strokes. Using sleep questionnaires, we investigated the prevalence of classified sleep disturbance and the influence of sleep disorders upon a stroke prognosis. METHODS: Patients with acute ischemic strokes or transient ischemic attacks (TIA) were included. We investigated the prevalence of sleep disturbance and the association of outcomes resulting from strokes. The National Institutes of Health Stroke Scale score at day 7 (NIHSS-7) and modified Rankin Scale score at month 3 (mRS-3) stood for short- and long-term outcomes. A series of questionnaires including all Korean versions of the Pittsburgh Sleep Quality Index (PSQI-K), Insomnia Severity Index (ISI-K), Epworth Sleepiness Scale (ESS-K), Berlin Questionnaire, Sleep Obstructive apnea score optimized for Stroke (SOS), Beck Depression Inventory-2, and Hospital Anxiety and Depression Scale were used. RESULTS: A total of 241 (mean age was 64.2 ± 11.9, 146 males; 60.6%) consecutive acute ischemic stroke patients, including 36 TIAs, were enrolled. The NIHSS score at admission, NIHSS-7, and mRS-3 were 3.26 ± 3.64, 1.72 ± 2.29, and 0.21 ± 0.82, respectively. PSQI-K ≥8.5 was reported in 79 subjects (32.8%), ISI-K ≥15.5 in 29 (12.0%), ESS-K ≥11 in 21 (8.7%), and SOS ≥11 in 48 (20.3%). The NIHSS-7 was associated with the SOS (standardized ß = 0.281, p < 0.001) and the mRS-3 with the ISI-K (standardized ß = 0.219, p = 0.001) and the SOS (standardized ß = 0.171, p = 0.011). CONCLUSIONS: Screening for and intervening in the sleep problems of stroke patients could improve their outcome. As sleep disturbances are associated with short-term and/or long-term outcomes of strokes, active screening and intervention for sleep disturbances after strokes are needed.

Perceived stress in patients with migraine: a case-control study.

BACKGROUND: Perceived stress is the most common trigger for migraine. The objective of this study was to examine the clinical significance of perceived stress in migraine patients. METHODS: This is a case-control study. Consecutive migraine patients who visited a tertiary care hospital were enrolled for this study. They completed self-reported questionnaires including Perceived Stress Scale (PSS), 12-item Allodynia Symptom Checklist (ASC-12), Migraine Disability Assessment Scale (MIDAS), Patient Health Questionnaire-9 (PHQ-9), Generalized Anxiety Disorder-7 (GAD-7), Epworth Sleepiness Scale (ESS), Insomnia Severity Index (ISI), and Migraine-Specific Quality of Life Questionnaire (MSQ). Degree of perceived stress in migraine patients was measured and compared to that in healthy controls. Predictors for perceived stress and their impact on quality of life (QOL) of migraine patients were also determined. RESULTS: A total of 227 migraine patients were eligible for this study, including 103 (45.4%) who had chronic migraine (CM). Mean PSS score was significantly (p < 0.05) higher in CM patients than that in controls after adjusting for education, depression, and anxiety. Although several factors were associated with PSS score, major predictors for PSS were GAD-7 score (ß = 0.358, p < 0.001), PHQ-9 score (ß = 0.304, p < 0.001), ISI score (ß = 0.154, p = 0.005), and CM (ß = -0.104, p = 0.027). There was an inverse relationship between PSS scores and three-dimensional scores of MSQ (p < 0.001). CONCLUSIONS: Chronic migraine is a critical factor for perceived stress. Perceived stress affects QOL of migraine patients.

Prediction of mortality and functional outcome from status epilepticus and independent external validation of STESS and EMSE scores.

BACKGROUND: Two clinical scoring systems, the status epilepticus severity score (STESS) and the epidemiology-based mortality score in status epilepticus (EMSE), are used to predict mortality in patients with status epilepticus (SE). The aim of this study was to compare the outcome-prediction function of the two scoring systems regarding in-hospital mortality using a multicenter large cohort of adult patients with SE. Moreover, we studied the potential role of these two scoring systems in predicting the functional outcome in patients with SE. METHODS: The SE cohort consisted of patients from the epilepsy centers of eight academic tertiary medical centers in South Korea. The clinical and electroencephalography data for all adult patients with SE from January 2013 to December 2014 were derived from a prospective SE database. The primary outcome variable was defined as in-hospital death. The secondary outcome variable was defined as a poor functional outcome, i.e., a score of 1-3 on the Glasgow Outcome Scale, at discharge. RESULTS: Among the 120 non-hypoxic patients with SE recruited into the study, 16 (13.3%) died in the hospital and 64 (53.3%) were discharged with a poor functional outcome. The receiver-operating characteristic (ROC) curve for prediction of in-hospital death based on the STESS had an area under the curve of 0.673 with an optimal cutoff value for discrimination (best match for both sensitivity (0.56) and specificity (0.70)) that was ≥ 4 points. The two combinations of elements of the EMSE system (EMSE-ALDEg and EMSE-ECLEg) predicted not only in-hospital mortality with the best match for sensitivity (more than 0.6) and specificity (more than 0.6), but also a poor functional outcome with the best match for both sensitivity (>0.7) and specificity (>0.6). STESS did not predict a poor functional outcome (area under the ROC, 0.581; P = 0.23). CONCLUSION: Although the EMSE is a clinical scoring system that focuses on individual mortality, we did not find differences between the EMSE and STESS in the prediction of in-hospital death. The EMSE was useful in predicting poor functional outcome, as it was significantly better than STESS.

Perceived stress and its predictors in people with epilepsy.

OBJECTIVE: Perceived stress in people with epilepsy (PWE) is one of the major precipitants for seizures. We investigated the degree of perceived stress in PWE and its predictors. We also aimed to reveal the interrelationships among the predictors. METHODS: This was a case-control study. Consecutive patients visiting a tertiary care epilepsy clinic completed self-reported questionnaires including the Perceived Stress Scale (PSS), Revised Stigma Scale (RSS), Korean version of the Neurological Disorders Depression Inventory for Epilepsy (K-NDDI-E), Generalized Anxiety Disorder - 7 (GAD-7), and short forms of the Patient-Reported Outcomes Measurement Information System - Sleep Disturbance (PROMIS-SD) and Patient-Reported Outcomes Measurement Information System - Sleep-Related Impairment (PROMIS-SRI) scales. RESULTS: The mean score of the PSS was significantly lower in patients with well-controlled epilepsy (WCE) and higher in those with uncontrolled epilepsy compared with controls. Although several factors including demographic, socioeconomic, psychosomatic, and epilepsy-related factors were associated with the PSS score, the strongest predictor for the PSS score was the K-NDDI-E score, followed by the PROMIS-SRI score, the GAD-7 score, and seizure control. Psychosomatic factors exerted both a direct effect on the PSS score and an indirect effect on the PSS score through seizure control. CONCLUSION: Rapid detection and appropriate management of psychiatric and sleep-related problems in PWE may lessen stress and aid in preventing further seizures.

Augmentation in restless legs syndrome patients in Korea.

PURPOSE: Augmentation has been known as the major complication of long-term dopaminergic treatment of restless legs syndrome (RLS). However, there have been no reports on the prevalence of augmentation in Korea. Thus, we aimed to assess the rate of augmentation and evaluate related factors in Korean RLS patients. METHODS: Ninety-four idiopathic RLS patients who have been treated over a period of at least 6 months were enrolled. Thirty subjects were treated with a dopamine agonist only, and 64 were treated with a dopamine agonist and alpha two delta ligands. We assessed the clinical characteristics of those RLS subjects and evaluated the rate of augmentation. Augmentation was assessed using the NIH criteria for augmentation by two RLS experts independently. RESULTS: Eleven subjects (11.7%) were classified as having definitive or highly suggestive clinical indication of augmentation. In comparing the augmentation group with the non-augmentation group, there were no significant differences of baseline clinical characteristics. Four (13.3%) of the dopamine agonists monotherapy group and seven (10.9%) of the combination therapy group were categorized as augmentation. There was no significant difference in the augmentation rate between these two groups. CONCLUSIONS: We found an 11.7% augmentation rate in Korean RLS subjects. There was no difference in the rate of RLS augmentation between the dopaminergic monotherapy group and the combined treatment group. It may be related with using a similar dosage of dopaminergic drugs.

Validation of the generalized anxiety disorder-7 in people with epilepsy: a MEPSY study.

The Generalized Anxiety Disorder-7 (GAD-7) is a valuable instrument to screen for anxiety in primary care patients. However, it has not been validated in people with epilepsy (PWE). Therefore, we validated the GAD-7 and examined its differential effect from adverse effects of antiepileptic drugs (AEDs) on the detection of anxiety in Korean PWE. Eligible patients who visited outpatient clinics in 4 tertiary care hospitals and 1 secondary care hospital underwent several instruments including the Mini International Neuropsychiatric Interview-Plus Version 5.0.0 (MINI-Plus 5.0.0), the Korean version of the Neurological Disorders Depression Inventory for Epilepsy (K-NDDI-E), the Korean version of the Liverpool Adverse Event Profile (K-LAEP), and the Quality of Life in Epilepsy-10 (QOLIE-10). Two hundred forty-three patients were enrolled in the study, and 51 (21.0%) patients had GAD by the MINI-Plus 5.0.0. Cronbach's α coefficient for the GAD-7 was 0.924. At a cutoff score of 6, the GAD-7 had a sensitivity of 92.2%, a specificity of 89.1%, a positive predictive value of 69.1%, and a negative predictive value of 97.7%. The GAD-7 score was well correlated with the K-NDDI-E score, the K-LAEP score, and the QOLIE-10 overall and subscale scores. The impact of adverse effects of AEDs on the GAD-7 was less than that on the K-NDDI-E. In conclusion, the GAD-7 is a reliable and valid screening tool for detecting GAD in PWE.

Topiramate increases the risk of valproic acid-induced encephalopathy.

Metabolic encephalopathy is a rare but serious complication of valproic acid (VPA) therapy that usually presents with impaired consciousness or increased seizure frequency. Although it has been suggested that topiramate (TPM) increases the risk of VPA-induced encephalopathy, the additional risk in patients receiving TPM therapy has not been evaluated. We reviewed all adult patients who took VPA between January 2005 and February 2009 at the Seoul National University Hospital and identified patients with VPA-induced encephalopathy based on clinical and electroencephalography (EEG) data. Information on sex, age, serum ammonia level, serum VPA level, liver function test, and EEG was collected from patient registry and medical data. We enrolled 8,372 patients who received VPA therapy and 1,236 patients who received VPA/TPM combination therapy. We identified 11 patients with VPA-induced encephalopathy (0.13%), 7 of whom received a combination therapy of VPA and TPM. The odds ratio of VPA-induced encephalopathy with TPM over that without TPM was 10.16. There were no significant differences in sex distribution, number of antiepileptic agents, ammonia level, VPA serum level, underlying diseases, dosage of VPA, duration of VPA treatment, treatment of encephalopathy, and outcomes between the two groups. Our study showed that the prevalence of VPA-induced encephalopathy is approximately 0.1% among patients treated with VPA and that the risk of this condition, although still low, can increase by approximately 10 times in the presence of TPM therapy. Based on these results, we suggest that TPM should be carefully used in patients receiving VPA treatment.

The long-term efficacy and safety of levetiracetam in a tertiary epilepsy centre.

AIM: To evaluate the long-term efficacy and safety of levetiracetam based on a large population of patients in a tertiary epilepsy centre. METHODS: All patients who received levetiracetam at the Seoul National University Hospital between January 2007 and March 2009 were evaluated. Patients who underwent brain surgery for seizure control or who had associated progressive disease were excluded from this study. The electronic medical records of these patients were reviewed retrospectively. RESULTS: A total of 568 patients were recruited, including 124 patients with generalised epilepsy. The mean duration of the follow-up period was 29.3 months. The seizure-free rate was 33.6% and was higher in patients with generalised epilepsy (51.6%) than patients with localisation-related epilepsy (28.6%). There was a strong correlation between initial response and dose-up response in 351 patients with increased dosage during the follow-up period. A total of 486 adverse events developed in 316 patients. The most common adverse event (24.3%) was irritability, which was associated with a high rate of drug discontinuation. Previous history of mood disorder was the only factor related to the development of irritability in patients using this medication. CONCLUSION: Levetiracetam was effective and safe as monotherapy and add-on therapy for partial and generalised epilepsy. The initial response to levetiracetam may provide useful information for predicting the response to increased dose of levetiracetam. However, the use of this medication was associated with a rate of irritability that was higher than expected in patients with a history of mood disorders.

Premature Mortality and Causes of Death Among People With Epilepsy: A Nationwide Population-Based Incident Cohort Study.

BACKGROUND AND OBJECTIVES: People with epilepsy (PWE) are at risk of premature death with considerable variability according to the study population. We aimed to estimate the risk and causes of death in PWE according to age, disease severity, disease course, comorbidities, and socioeconomic status in Korea. METHODS: We conducted a nationwide population-based retrospective cohort study using the National Health Insurance database linked with the national death register. Newly treated PWE from 2008 to 2016 who were identified by antiseizure medication (ASM) prescriptions and diagnostic codes for epilepsy/seizure were included and observed until 2017. We assessed all-cause and cause-specific crude mortality rates and standardized mortality ratios (SMRs). RESULTS: Among 138,998 PWE, 20,095 deaths were identified, and the mean follow-up period was 4.79 years. The SMR was 2.25 in the overall group of PWE, with a higher value in the younger age group at diagnosis and a shorter time interval after diagnosis. The SMR in the monotherapy group was 1.56, while that in the group with 4 or more ASMs was 4.93. PWE without any comorbidities had an SMR of 1.61. PWE who were rural residents had a higher SMR than those who were urban residents (2.47 vs 2.03, respectively). The causes of death among PWE were cerebrovascular disease (18.9%, SMR 4.50), malignant neoplasms outside the CNS (15.7%, SMR 1.37), malignant neoplasms of the CNS (6.7%, SMR 46.95), pneumonia (6.0%, SMR 2.08), and external causes (7.2%, SMR 2.17), including suicide (2.6%, SMR 2.07). Epilepsy itself and status epilepticus accounted for 1.9% of the overall death. The excess mortality associated with pneumonia and external causes was persistently high, whereas the excess mortality associated with malignancy and cerebrovascular diseases tended to decrease with increasing time since diagnosis. DISCUSSION: This study showed excess mortality in PWE, even in those without comorbidities and those receiving monotherapy. Regional disparities and sustained risks of deaths from external causes over 10 years imply potential points of intervention. In addition to active control of seizures, education about injury prevention, monitoring for suicidal ideation, and efforts to improve accessibility to epilepsy care are all required to reduce mortality.

Sleep and Circadian Rhythm in Relation to COVID-19 and COVID-19 Vaccination-National Sleep Survey of South Korea 2022.

BACKGROUND: Currently, information on sleep and circadian patterns in relation to COVID-19 or vaccination remains limited. We aimed to investigate sleep and circadian patterns according to history of COVID-19 and COVID-19 vaccination side effects. METHODS: We used data from the National Sleep Survey of South Korea 2022, a nationwide cross-sectional population-based survey regarding sleep-wake behaviors and sleep problems among Korean adults. Analysis of covariance (ANCOVA) and logistic regression analyses were performed to explore the different sleep and circadian patterns according to the history of COVID-19 or self-reported side effects of the COVID-19 vaccination. RESULTS: The ANCOVA showed that individuals with a history of COVID-19 presented a later chronotype than individuals without a history of COVID-19. Individuals who had experienced vaccine-related side effects had a shorter sleep duration, poorer sleep efficiency, and worse insomnia severity. Multivariable logistic regression analysis showed a later chronotype related to COVID-19. A short sleep duration, poorer sleep efficiency, and worse insomnia severity were associated with self-reported side effects of the COVID-19 vaccination. CONCLUSIONS: Individuals who recovered from COVID-19 had a later chronotype than those without a history of COVID-19. Individuals who had experienced vaccine-related side effects presented with poorer sleep than those without side effects.

Trends in Prescribing of Antiseizure Medications in South Korea: Real-World Evidence for Treated Patients With Epilepsy.

BACKGROUND AND PURPOSE: We investigated the trends in the prescribing of antiseizure medication (ASM) over a 9-year period, and provide real-world data regarding ASM prescriptions of patients with epilepsy in South Korea. METHODS: This study used data in the Korean National Health Information Database for the period from 2009 to 2017. We included 18 oral ASMs, which were classified into older and newer ASMs based on them first becoming available on the market before or after 1991, respectively. The annual trends in ASM prescriptions were plotted over the 9-year study period, and changes in these trends were evaluated as average annual percentage changes (AAPCs) using Poisson regression. Age- and sex-stratified analyses were also conducted. RESULTS: Overall, the proportion of prescriptions involving polytherapy with three or more ASMs increased from 10.08% in 2009 to 10.99% in 2017 (AAPC=0.9%, p<0.001) over the 9-year study period. Among monotherapies, although valproate (VPA) was the most frequently prescribed ASM, the prescription rate of levetiracetam (LEV) steadily increased regardless of age and sex over the study period. The monotherapy prescription trends differed depending on age and sex. In the five most frequently used ASM combination regimens, the prescription rates of VPA/LEV, LEV/oxcarbazepine, and LEV/lamotrigine regimens showed increasing tendencies. In contrast, prescription rates for all combined regimens of older ASMs declined over time in all age groups. CONCLUSIONS: This is the first epidemiological study of the changes in prescription trends for ASM in South Korea based on nationwide data from 2009 to 2017. We found progressive increases in the use of newer ASMs for both monotherapy and duotherapy, and for polytherapy with three or more ASMs over the 9-year study period.

Mortality, Disability, and Prognostic Factors of Status Epilepticus: A Nationwide Population-Based Retrospective Cohort Study.

BACKGROUND AND OBJECTIVES: The outcome of status epilepticus (SE) largely varies depending on clinical characteristics. Risk stratification is necessary for tailoring the aggressiveness of treatment and predicting outcomes of individual patients with SE. In this study, we assessed differences in mortality, neurologic disability, and prognostic factors associated with SE across sociodemographic and clinical characteristics. METHODS: We conducted a nationwide population-based retrospective cohort study using the National Health Insurance Service (NHIS) database linked with the national death and disability registries. SE was identified from admission or emergency department visits using a diagnostic code of G41 from the International Classification of Diseases, 10th Revision. Individuals with new-onset SE that occurred from January 1, 2010, to December 31, 2018, were included. Active epilepsy, refractoriness of SE, potential etiology, and comorbidities were ascertained by diagnostic codes and/or prescription records from the NHIS database as potential prognostic factors. Outcomes included 30-day and 1-year mortality and neurologic disabilities after SE. Prognostic factors for mortality were assessed by the Cox regression hazard model. We performed a subgroup analysis according to age: pediatric SE (age <20 years) and adult SE (age ≥20 years). RESULTS: A total of 33,814 patients with new-onset SE were included (6,818 children/adolescents and 26,996 adults). The 30-day mortality was 8.5% (1.8% in pediatric SE and 10.2% in adult SE), and the 1-year mortality was 25.1% (4.6% in pediatric SE and 30.3% in adult SE). Overall, 10.7% of patients newly acquired neurologic disabilities after SE, with the highest incidence in children aged 5-9 years (21.3%). Intractable epilepsy developed in 0.8% of entire SE. Old age, presence of acute etiology, and refractoriness were poor prognostic factors for mortality in both pediatric and adult SE. Male sex, low economic status, no active epilepsy, and comorbidities were additional factors for a poor prognosis in adults. DISCUSSION: New-onset SE was associated with substantial mortality and disability. Although SE-related mortality was higher in adults, disabilities developed more commonly in children and adolescents. The major determinants of mortality differed between pediatric and adult SE.

Axonal conduction block at intermediate nerve segments in pure motor Guillain-Barré syndrome.

The pathophysiology of axonal Guillain-Barré syndrome (GBS) is not simple axonal degeneration, but includes reversible conduction failure. Acute motor axonal neuropathy (AMAN) and acute motor conduction block (CB) neuropathy are the two subtypes of pure motor axonal GBS, but their nosologic boundary is still in debate. We investigated clinical and electrophysiological features of 21 consecutive patients with GBS in Korea. Analysis was focused on the presence of CB at intermediate nerve segments (iCB) in pure motor GBS, and its serial changes during the acute phase of disease. Pure motor GBS was common (81%), and iCB was observed in 12 patients with pure motor GBS. Clinical features of pure motor GBS with iCB were distinct from sensorimotor GBS, but similar to pure motor GBS without iCB, characterized by frequent preceding diarrhea, uncommon cranial nerve palsy, and fast recovery. The iCB was not restricted to common entrapment sites, and the distal segments were also commonly involved in the nerves with iCB. The temporal course of iCB was marked by a rapid and often disproportionate increase of proximal and distal amplitudes without remyelinating slow components. Clinical and electrophysiological features of pure motor GBS in patients with iCB suggest that acute motor CB neuropathy may constitute a spectrum of axonal GBS, sharing a common pathomechanism with AMAN.

Increasing Trends in the Incidence and Prevalence of Epilepsy in Korea.

BACKGROUND AND PURPOSE: There have been few reports on recent trends in the occurrence of epilepsy. The aim of this study was to estimate the incidence and prevalence of epilepsy and analyze their annual trends in Korea over the period 2009-2017. METHODS: This nationwide population-based study was carried out using the National Health Insurance Service of Korea database. A prevalent case was defined as one of a patient receiving a prescription of anticonvulsants under the diagnostic codes for epilepsy or seizure. An incident case was ascertained by confirming the absence of any epilepsy-related diagnostic codes and anticonvulsant prescription for 2 years or more before the operational definition for a prevalent case was met. Alternative operational definitions for epilepsy were tested. The temporal trends of the incidence and prevalence of epilepsy were analyzed using a Poisson regression model, and are expressed as average annual percentage changes (AAPCs). RESULTS: The incidence of epilepsy increased from 28.7/100,000 persons in 2009 to 35.4/100,000 persons in 2017. The prevalence increased gradually from 3.4/1,000 persons in 2009 to 4.8/1,000 persons in 2017. These increasing trends were more evident among elderly subjects aged ≥75 years and in those who had codes for epilepsy or seizure as an additional diagnosis. Age standardization revealed a less prominent but still increasing trend in both incidence (AAPC=0.48%) and prevalence (AAPC=3.11%). CONCLUSIONS: There have been increasing trends in both the prevalence and incidence of epilepsy in Korea between 2009 and 2017. This finding appears to be related to societal aging and the high incidence of symptomatic epilepsy in the elderly population.

Survey on Antiepileptic Drug Therapy in Patients with Drug Resistant Epilepsy.

BACKGROUND AND PURPOSE: Individualized anti-epileptic drug (AED) selection in patient with epilepsy is crucial. However, there is no unified opinion in treating patients with drug resistant epilepsy (DRE). This survey aimed to make a consolidate consensus with epileptologists' perspectives of the treatment for Korean DRE patients by survey responses. METHODS: The survey was conducted with Korean epilepsy experts who have experience prescribing AEDs via e-mail. Survey questionnaires consisted of six items regarding prescription patterns and practical questions in treating patients with DRE in Korea. The research period was from February 2021 to March 2021. RESULTS: The survey response rate was 83.3% (90/108). Most (77.8%) of the responders are neurologists. The proportion of patients whose seizures were not controlled by the second AED was 26.9%. The proportion of patients who had taken five or more AEDs is 13.9%, and those who are currently taking five or more AEDs are 7.3%, of which 54.5% and 37.9% reported positive effects on additional AED, respectively. The majority (91.1%) of respondents answered that the mechanism of action was the top priority factor when adding AED. Regarding data priority, responders considered that expert opinion should have the top priority, followed by clinical experiences, reimbursement guidelines and clinical evidence. Responders gave 64.9 points (range from 0 to 100) about overall satisfaction on reimbursement system of Health Insurance Review and Assessment Service for AED. CONCLUSIONS: This study on AED therapy for DRE patients is the first nationwide trial in Korean epilepsy experts. In five drug failure, the top priorities on AED selection are mechanism of action and expert opinion. These findings might help to achieve consensus and recognize the insight on optimal therapy of AED in DRE.

A case of NMDAR encephalitis treated in the third trimester - novel arterial spin labeling findings and a review of literature.

Diagnosis and treatment of anti-NMDAR encephalitis during pregnancy are challenging. We report a case of anti-NMDAR encephalitis in a 28-year-old, 24 weeks pregnant woman. Her brain magnetic resonance imaging was normal, but arterial spin labeling (ASL) showed increased cerebral blood flow in the right insula and temporal area. She was successfully treated with emergent cesarean section and immunotherapies including intravenous immunoglobulin, steroids, and rituximab. The newborn was healthy and she recovered fully. Early suspicion and proper management is important in treatment of anti-NMDAR encephalitis during pregnancy. ASL can help diagnosing anti-NMDAR encephalitis during pregnancy.