Evaluation of Sleep Disturbance in Alopecia Areata through Questionnaire: Pittsburgh Sleep Quality Index as a Reasonable Tool.

BACKGROUND: Alopecia areata (AA) is common non-scarring hair loss disease. Sleep distrubance has been regarded as a triggering or aggravating factor for AA. However, objective evaluation of sleep disturbance and its clinical effect on AA has not been clearly demonstrated. OBJECTIVE: This study investigated objective sleep evaluation tool for AA patients and their clinical correlation. METHODS: Patients presenting with new-onset AA or recurrences of pre-existing AA were included, and those who reported sleep disturbance in the preliminary survey were designated as the sleep disturbance group (SD group). Sleep quality was investigated for them using three self-administered questionnaires: Pittsburgh Sleep Quality Index (PSQI), Insomnia Severity Index (ISI), and Epworth Sleep Scale (ESS). Demographic information and clinical features of AA were analyzed according to sleep quality. RESULTS: A total of 400 participants were enrolled, and 53 were categorized into the SD group. The incidence of stressful events was significantly higher in the SD group (54.7%) than in the non-SD group (25.1%) (p<0.001). Based on the PSQI, 77.3% of participants were objective poor sleepers (score of 5 or more), and they showed a significantly higher incidence of stressful events compared to good sleepers (p=0.019). The proportion of poor sleepers was significantly lower in patients with mild AA (S1) than in those with moderate to severe AA (S2~S5) (p=0.045). CONCLUSION: This study demonstrated a positive correlation among stress, SD, and AA. The degree of SD was objectively represented by the PSQI score, showing different scores according to AA severity.

Unexpected Resolution of Obstructive Sleep Apnea after Nasal Surgery in a Patient Nonadherent to Continuous Positive Airway Pressure Therapy.

Continuous positive airway pressure (CPAP) is recommended as first-line therapy in cases of moderate-to-severe obstructive sleep apnea (OSA). Continuous positive airway pressure (CPAP) prevents upper airway obstruction and abolishes apnea or hypopnea events during sleep. But up to 50% of OSA patients may be nonadherent to CPAP due to various causes, including nasal obstruction. Nasal surgery may improve CPAP compliance in some OSA patients but is not regarded as OSA therapy. Here, I report a patient non-adherent to CPAP whose sleep apnea resolved unexpectedly after nasal surgery to increase adherence to CPAP.

Atypical head banging developed in teens persisting into adulthood as sleep-related rhythmic movement disorder.

Head banging is one subtype of sleep-related rhythmic movement disorder, characterized by stereotyped, repetitive rhythmic movements, such as lifting the head or entire upper body, banging the frontal area hard on the pillow, or slamming the occiput against a headboard. An atypical form of head banging with punching or slapping the head with a hand is extremely rare, with only 4 such cases reported so far. Herein, we present a young adult male who, since his early teens, has had atypical head banging, also called head-slapping, and discuss the neuropsychological and polysomnographic findings and review the literature. CITATION: Ji K-H, Kang M-R, Kim SJ. Atypical head banging developed in teens persisting into adulthood as sleep-related rhythmic movement disorder. J Clin Sleep Med. 2023;19(5):999-1001.

Correlations with REM sleep behavior disorder severity in isolated rapid eye movement sleep behavior disorders patients.

OBJECTIVES: The pathogenesis of isolated rapid eye movement sleep behavior disorders (iRBD) is poorly understood. The severity of RBD may reflect its pathogenesis. METHODS: We compared motor function and non-motor symptoms (NMSs) between iRBD patients and healthy volunteers. We correlated motor function, NMSs, and striatal dopaminergic activity with RBD severity using video-polysomnography. RESULTS: Twenty-one iRBD patients and 17 controls participated. The Unified Parkinson's Disease Rating Scale part III scores were higher in patients compared to controls (p < 0.001). There was no difference in upper extremity function between patients and controls (right, p = 0.220; left, p = 0.209), but gait was slower in iRBD patients (walking time, p < 0.001; number of steps, p < 0.001). The mean value of the Korean version of the Mini-Mental State Exam and Clinical Dementia Rating were lower in patients (p = 0.006, p = 0.003, respectively). Patients with were also more depressed (p = 0.002), had decreased olfactory function (p < 0.001), reported more frequent sleep/fatigue episodes (p < 0.001), worse attention/memory capacity (p < 0.001), gastrointestinal problems (p = 0.009), urinary problems (p = 0.007), and pain (p = 0.083). Further, iRBD patients reported more frequent sleep-related disturbances (p = 0.004), but no difference in daytime sleepiness (p = 0.663). Disease severity was correlated with pain (r = 0.686, p = 0.002) and visuospatial function (r= -0.507, p = 0.038). There were no correlations between RBD severity and striatal dopaminergic activities (p > 0.09). CONCLUSIONS: iRBD is a multisystem neurodegenerative disorder, and gait abnormalities may be a disease characteristic, possibly related to the akinetic-rigid phenotype of Parkinson's disease. The correlation between pain/visuospatial dysfunction and RBD severity may be related to its pathogenesis.

Acceptability of the 8-case objective structured clinical examination of medical students in Korea using generalizability theory: a reliability study

PURPOSE: This study investigated whether the reliability was acceptable when the number of cases in the objective structured clinical examination (OSCE) decreased from 12 to 8 using generalizability theory (GT). METHODS: This psychometric study analyzed the OSCE data of 439 fourth-year medical students conducted in the Busan and Gyeongnam areas of South Korea from July 12 to 15, 2021. The generalizability study (G-study) considered 3 facets­students (p), cases (c), and items (i)­and designed the analysis as p×(i:c) due to items being nested in a case. The acceptable generalizability (G) coefficient was set to 0.70. The G-study and decision study (D-study) were performed using G String IV ver. 6.3.8 (Papawork, Hamilton, ON, Canada). RESULTS: All G coefficients except for July 14 (0.69) were above 0.70. The major sources of variance components (VCs) were items nested in cases (i:c), from 51.34% to 57.70%, and residual error (pi:c), from 39.55% to 43.26%. The proportion of VCs in cases was negligible, ranging from 0% to 2.03%. CONCLUSION: The case numbers decreased in the 2021 Busan and Gyeongnam OSCE. However, the reliability was acceptable. In the D-study, reliability was maintained at 0.70 or higher if there were more than 21 items/case in 8 cases and more than 18 items/case in 9 cases. However, according to the G-study, increasing the number of items nested in cases rather than the number of cases could further improve reliability. The consortium needs to maintain a case bank with various items to implement a reliable blueprinting combination for the OSCE.

Comparing the cut score for the borderline group method and borderline regression method with norm-referenced standard setting in an objective structured clinical examination in medical school in Korea.

PURPOSE: Setting standards is critical in health professions. However, appropriate standard setting methods do not always apply to the set cut score in performance assessment. The aim of this study was to compare the cut score when the standard setting is changed from the norm-referenced method to the borderline group method (BGM) and borderline regression method (BRM) in an objective structured clinical examination (OSCE) in medical school. METHODS: This was an explorative study to model of the BGM and BRM. A total of 107 fourth-year medical students attended the OSCE at seven stations with encountering standardized patients (SPs) and one station with performing skills on a manikin on 15 July 2021. Thirty-two physician examiners evaluated the performance by completing a checklist and global rating scales. RESULTS: The cut score of the norm-referenced method was lower than that of the BGM (p<0.01) and BRM (p<0.02). There was no significant difference in the cut score between the BGM and BRM (p=0.40). The station with the highest standard deviation and the highest proportion of the borderline group showed the largest cut score difference in standard setting methods. CONCLUSION: Prefixed cut scores by the norm-referenced method without considering station contents or examinee performance can vary due to station difficulty and content, affecting the appropriateness of standard setting decisions. If there is an adequate consensus on the criteria for the borderline group, standard setting with the BRM could be applied as a practical and defensible method to determine the cut score for OSCE.

Renaming Korean epilepsy: Where are we?

OBJECTIVE: The Korean name of epilepsy was renamed in 2011 to reflect the modern scientific evidence and to avoid misconceptions of epilepsy. However, the degree of dissemination of the new name of epilepsy had not been studied in Korea. To determine the degree of dissemination of the new name, we retrieved the relative Internet search volume of the new name and the old name using Naver, a dominant Internet search engine in Korea. METHODS: The relative search volume is referred to as the Naver Trend Index (NTI). Naver DataLab provides the information on queries only from January 2016, and we downloaded a total of 846 daily NTI for the new name and old name for Korean epilepsy from January 1, 2016 to April 25, 2018. The proportions of NTI for the new name over the old name were computed. A primary hypothesis was that the ratio of Internet search volume of the new name exceeds that of the old name in 2018. A secondary hypothesis was that the new name had been continuously replacing the old name. RESULTS: The old name was more frequently used for Internet search. We found that about one-fourth (26%) of the Internet searches for Korean epilepsy were carried out with the new name in Naver DataLab between January 2016 and April 2018. The mean log ratio (NTI new/NTI old) in 2018 was -0.87, which indicates that the new name was used in 29.6% of the total search volume. In all ages, robust regression revealed log (NTI new, day/NTI old, day) = -8.79 + 0.00037 ∗ day, P < 0.001. The annual means of log (NTI new/NTI old) were the lowest in the 20s but increased with age and were higher in females than in males except for the age groups under 19 and over 60 years. CONCLUSIONS: The ratio of Internet search volume of the new name over the old name was 1:2.4 (29.6% of the total search volume) in 2018. The new name was used in 26% of the total Internet searches in the search period. The degree of dissemination of the new name seems to increase with age and with females being more likely to use the new name in Internet searches versus males. The implementation and utilization of the new name by the public is a slow process.

Wilson's disease with cognitive impairment and without extrapyramidal signs: improvement of neuropsychological performance and reduction of MRI abnormalities with trientine treatment.

Extrapyramidal signs are neurological dysfunction commonly associated with Wilson's disease (WD). In addition, cognitive dysfunction has been reported in the early stages of WD. In this report, we describe a 49-year-old woman presenting with memory impairments and without Parkinsonian or extrapyramidal signs. She was diagnosed with WD based on the presence of Kayser-Fleischer rings around the irises of her eyes and two ATP7B gene mutations, R778L at exon 8 and A874V at exdyon 11. Serial magnetic resonance imaging analysis and neuropsychological tests showed improvements following treatment with trientine.

Analysis among cognitive profiles and gray matter volume in newly diagnosed Parkinson's disease with mild cognitive impairment.

PURPOSE: To analyze the characteristics of neuropsychological profiles and gray matter volume in de novo, drug-naïve Parkinson's disease with mild cognitive impairment (PD-MCI). METHODS: Fifty-two newly diagnosed PD patients were assessed with neuropsychological testing, and PD-MCI was defined in patients with at least one or more abnormal cognitive test domains. PD with normal cognition (PD-NC) did not meet the criteria for PD-MCI or PD with dementia. Brain MRI scans was acquired from the patients and healthy controls (HC). The imaging processing and analysis of the gray matter (GM) volume were performed platform to determine the difference between PD-MCI and PD-NC. RESULTS: There were no differences of motor subscores between PD-MCI and PD-NC. The clinical dementia rating, global deterioration scale, and verbal memory were significantly worse in PD-MCI than in PD-NC. GM volume loss was observed in the right hippocampus, right cuneus, and right precuneus in PD-NC compared to in the HC. PD-MCI had significantly decreased GM volume in the bilateral temporal and frontal areas compared with that of the HC. The GM volume was significantly decreased in the right temporal pole, left precuneus, medial frontal and posterior cingulated gyrus in PD-MCI compared with PD-NC. CONCLUSIONS: Clinical dementia ratings and global deterioration scales could differentiate PD-MCI from PD-NC. Verbal memory impairment is characterized as a cognitive deficit of de novo PD-MCI and is associated with the posterior cortical area.

CPAP therapy reverses weakness of myasthenia gravis: role of obstructive sleep apnea in paradoxical weakness of myasthenia gravis.

Fatigability after exercise and restoration of strength by rest are clinical hallmarks of myasthenia gravis (MG). These unique features are originated from a reduced safety factor, which is related to a defect of acetylcholine receptor, and post-exercise exhaustion of neuromuscular junction transmission. Therefore, most patients with MG feel recharged muscle strength and show normal muscle power after nocturnal sleep. As such, worsening of MG symptoms during nighttime is usual. However, in very rare cases, some patients with MG complain fatigability and weakness in the morning, so called "paradoxical weakness (PW)." We report two MG patients with typical PW, diagnosed with OSA by polysomnography. CPAP therapy successfully improved their morning symptoms and quality of life. So far, the detailed mechanism of PW in MG is unknown; however, our report highlights the possible role of sleep disorders in developing a PW in MG and the therapeutic target for life quality of MG.

Initial response to antiepileptic drugs in patients with newly diagnosed epilepsy.

This study aimed to identify factors predicting the response to antiepileptic drugs in patients with newly diagnosed epilepsy. We prospectively studied 176 patients with newly diagnosed epilepsy. Patients were included if they had a history of two or more clinically definite unprovoked seizures, or had a definite epileptic focus on MRI or epileptiform discharges on electroencephalography if they had suffered only one seizure. The primary endpoint was seizure freedom during the initial 6 months of antiepileptic drug treatment. The secondary endpoint was the time to the first seizure during the maintenance period of antiepileptic drug treatment. A total of 100 patients were included, and seizure freedom for 6 months was achieved in 73 patients. The response to antiepileptic drugs was significantly lower in patients with early age at seizure onset (⩽ 16 versus >16 years old, odds ratio=4; 95% confidence interval [CI] 1.5-12.9; relative risk=1.4; 95% CI 1.1-1.8). In addition, the time to the first seizure during the maintenance period was significantly earlier in patients with age at seizure onset ⩽ 16 years compared with those with age at seizure onset >16 years on the Kaplan-Meier survival analysis (p=0.011). Early age at seizure onset is an important factor influencing the response to antiepileptic drugs in patients with newly diagnosed epilepsy.

Expansion of the clinicopathological and mutational spectrum of Perry syndrome.

BACKGROUND: Perry syndrome (PS) caused by DCTN1 gene mutation is clinically characterized by autosomal dominant parkinsonism, depression, severe weight loss, and hypoventilation. Previous pathological studies have reported relative sparing of the cerebral cortex in this syndrome. Here, we characterize novel clinical and neuroimaging features in 3 patients with PS. METHODS: (18)F-fluorinated N-3-fluoropropyl-2-ß-carboxymethoxy-3-ß-(4-iodophenyl) nortropane ([(18)F]FP-CIT) PET, [(18)F]fluorodeoxyglucose PET, or volumetric MRI was performed in probands, and imaging data were analyzed and compared with those of control subjects. RESULTS: We identified 2 novel mutations of DCTN1. Oculogyric crisis that presented before levodopa treatment was observed in 1 case. One patient had supranuclear gaze palsy. In 2 cases, [(18)F]FP-CIT showed marked loss of dopamine transporter binding with only mild parkinsonism. Areas of hypometabolism or cortical thickness change were observed in dorsolateral frontal, anterior cingulate, lateral temporal, and inferior parietal cortices. CONCLUSION: Oculomotor manifestations are not uncommon in PS. Neuroimaging studies suggest involvement of the frontotemporoparietal cortex, which may be the clinical correlate of apathy and depression, as well as pathological changes in subcortical structures.

Cerebral blood flow abnormalities in patients with neurally mediated syncope.

The aim of this study is to investigate regional cerebral blood flow (rCBF) in patients with syncope. We compared brain single photon emission computed tomography (SPECT) images of neurally mediated syncope patients with those of age/sex matched healthy volunteers. (99m)Tc-ethylcysteinate dimer (ECD) brain SPECT was performed in 35 patients (M/F = 17/18, mean 36.6 years) with syncope during the asymptomatic period, and in 35 healthy volunteers. For statistical parametric mapping (SPM) analysis, all SPECT images were spatially normalized to the standard SPECT template and then smoothed using a 14-mm full width at half maximum Gaussian kernel. The mean duration of syncope history was 4.9 years and the mean number of syncope episodes was 6.3. In all patients, syncope or presyncope episodes occurred during head-up tilt tests, and all were the vasodepressive type. SPM analysis of brain SPECT images showed significantly decreased rCBF in the right anterior insular cortex, left parahippocampal gyrus, bilateral fusiform gyri, bilateral middle and inferior temporal gyri, left lingual gyrus, bilateral precuneus and bilateral posterior lobes of the cerebellum in syncope patients at a false discovery rate corrected p < 0.05. There were no brain regions that showed increased rCBF in syncope patients. Furthermore, we found a negative correlation between the total number of syncopal episodes and the rCBF of the right prefrontal cortex, and between the duration of syncope history and the rCBF of the right cingulate gyrus at uncorrected p < 0.001. Decreases of rCBF in multiple brain regions may be responsible for autonomic dysregulation and improper processing of emotional stress in neurally mediated syncope patients, and frequent syncope episodes may lead to frontal dysfunction.

The effect of modafinil on cortical excitability in patients with narcolepsy: a randomized, placebo-controlled, crossover study.

OBJECTIVE: To investigate the effect of modafinil on cortical excitability in patients with narcolepsy using transcranial magnetic stimulation (TMS). METHODS: Nineteen drug-naïve narcolepsy patients with cataplexy (10 males, 9 females, and mean age 28.5 years) and 25 age- and sex-matched healthy controls were recruited. In this double-blind, randomized, crossover study, patients and controls received a single dose of 400mg modafinil or placebo. Modafinil and placebo administrations were separated by a 2-week washout period. TMS parameters, such as resting motor thresholds (RMT), motor-evoked potential (MEP) amplitudes, cortical silent periods (CSP), short-interval intracortical inhibition (SICI), and intracortical facilitation (ICF), were measured before and 3h after administering modafinil or placebo. The differences of TMS parameters were statistically tested between patients and controls and between before and after modafinil or placebo administration. RESULTS: Narcolepsy patients had significantly increased CSP durations compared to controls (independent t-test, P<0.05), indicating decreased excitability of cortical networks in human narcolepsy. In patients after modafinil administration, MEP amplitudes, SICI, and ICF increased, and CSP duration shortened significantly, meaning enhanced motor excitability, whereas in controls modafinil did not change TMS parameters significantly. Placebo administration did not affect TMS parameters both in patients or controls. CONCLUSIONS: Narcolepsy patients with cataplexy showed decreased cortical excitability than normal healthy controls. Single dose modafinil significantly increased motor excitability in narcolepsy patients but had no effect in healthy controls.

Increased circulating endothelial microparticles and carotid atherosclerosis in obstructive sleep apnea.

BACKGROUND AND PURPOSE: Endothelial impairment is a linking mechanism between obstructive sleep apnea (OSA) and cardiovascular diseases. Profiles of endothelial microparticles (EMPs) and endothelial progenitor cells (EPCs) reflect the degree of endothelial impairment. The aims of this study were to measure the levels of EMPs and progenitor cells in OSA, determine the correlations between these factors and OSA severity and the degree of atherosclerosis, and document any changes in these factors after therapy. METHODS: Subjects with (n=82) and without (n=22) OSA were recruited prospectively. We measured the number of colony-forming units (CFU) in cell culture as the endothelial progenitor cell index, and the number of EMPs using flow cytometry with CD31 [platelet endothelial cell adhesion molecule (PECAM)], CD42 (platelet glycoprotein), annexin V, and CD62E (E-selectin) antibodies at baseline and after 4-6 weeks of continuous positive airway pressure (CPAP) therapy. Carotid intima-media thickness (IMT) was regarded as a marker of atherosclerosis. RESULTS: The levels of PECAM(+)CD42(-) (p<0.001), PECAM(+)annexin V(+) (p<0.001), and E-selectin(+) microparticles (p=0.001) were higher in OSA subjects than in non-OSA subjects. The number of CFU did not differ between the two groups. OSA severity independently predicted the levels of PECAM(+)CD42(-) (p=0.02) and PECAM(+)annexin V(+) (p=0.004). Carotid IMT was correlated with OSA severity (p<0.001), PECAM(+)CD42(-) (p=0.03), and PECAM(+)annexin V(+) (p=0.01). Neither OSA severity nor carotid IMT was correlated with either the number of CFU or E-selectin(+). CPAP therapy decreased the occurrence of E-selectin(+) (p<0.001) in 21 of the OSA subjects, but had no effect on the other microparticles of the number of CFU. CONCLUSIONS: OSA led to the overproduction of EMPs, which moderately correlated with OSA severity and the degree of atherosclerosis, and partly responded to therapy. The endothelial impairment might contribute to future cardiovascular events.

Zonisamide changes unilateral cortical excitability in focal epilepsy patients.

BACKGROUND AND PURPOSE: To evaluate changes in cortical excitability induced by zonisamide (ZNS) in focal epilepsy patients. METHODS: Twenty-four drug-naїve focal epilepsy patients (15 males; overall mean age 29.8 years) were enrolled. The transcranial magnetic stimulation parameters obtained using two Magstim 200 stimulators were the resting motor threshold, amplitude of the motor-evoked potential (MEP), cortical silent period, short intracortical inhibition, and intracortical facilitation. These five transcranial magnetic stimulation parameters were measured before and after ZNS, and the findings were compared. RESULTS: All 24 patients were treated with ZNS monotherapy (200-300 mg/day) for 8-12 weeks. After ZNS, MEP amplitudes decreased (-36.9%) significantly in epileptic hemispheres (paired t-test with Bonferroni's correction for multiple comparisons, p<0.05), whereas the mean resting motor threshold, cortical silent period, short intracortical inhibition, and intracortical facilitation were unchanged (p>0.05). ZNS did not affect cortical excitability in nonepileptic hemispheres. CONCLUSIONS: These findings suggest that ZNS decreases cortical excitability only in the epileptic hemispheres of focal epilepsy patients. MEP amplitudes may be useful for evaluating ZNS-induced changes in cortical excitability.

Zolpidem-induced sleep-related eating disorder.

An association between zolpidem administration and sleep-related eating disorder (SRED) has been suggested. The authors observed zolpidem-induced SRED in restless legs syndrome (RLS). With the review of previous reports, we identified a common occurrence of RLS in zolpidem-induced SRED.

Severe obstructive sleep apnea syndrome with symptomatic daytime bradyarrhythmia.

We report a case of severe obstructive sleep apnea (OSA) in a patient with symptomatic daytime cardiac bradyarrhythmia. Continuous positive airway pressure therapy prevented atrioventricular blocks that emerged after cardiac pacing for sick sinus syndrome. OSA could be associated with daytime bradyarrhythmia.