Atypical Paroxysmal Kinesigenic Dyskinesia with Paroxysmal Exercise-induced Dyskinesia

Paroxysmal kinesigenic dyskinesia (PKD) is a diagnostic term for transient, involuntary abnormal movements triggered by sudden motions. The treatment for PKD differs from other paroxysmal dyskinesias, as it notably responds well to sodium channel blockers. We report a case of atypical PKD, coupled with paroxysmal exercise-induced dyskinesia (PED). Both PKD and PED in this patient showed a good response to oxcarbazepine. This case could be clinical evidence that paroxysmal dyskinesias could potentially be regarded as a spectrum disorder with overlapping features.

A Study on the Evaluation of Brainstem Dysfunction in Rapid Eye Movement Sleep Behavior Disorder Using Video Nystagmography

Background@#This study aimed to differentiate video nystagmography (VNG) characteristics, including the video head impulse test (vHIT), in patients with idiopathic rapid eye movement behavior disorder (RBD) from healthy controls, which is considered a precursor to degenerative diseases. @*Methods@#One hundred eighty-five patients underwent overnight polysomnography (PSG) and VNG. Based on overnight PSG, 27 patients with RBD or REM sleep without atonia (RWA) and AHI<15 were categorized into the RBD group, 34 patients with RBD/RWA and AHI≥15 were grouped into the combined group. Sixty patients with AHI≥15 and no RBD/RWA were included in the obstructive sleep apnea (OSA) group, and 64 negative participants were assigned to the control group. In VNG, we measured the gain of vHIT in each canal, with the latency, amplitude, and velocity of horizontal saccades and smooth pursuit. We compared the results between groups using ANOVA, after normalization and adjustment for age and sex. @*Results@#The gain of vHIT in the left horizontal canal was decreased in the RBD group, but it was more pronounced in the OSA group. Elevated gain of the left posterior canal was seen in the RBD group, but technical errors were attributable. The RBD group displayed prolonged latency of saccade on the left side and slowed saccade on the right side, but these were statistically insignificant. @*Conclusions@#The VNG study revealed differences between the sleep disorders, potentially reflecting brainstem function in each disorder. However, these differences lacked statistical significance. We anticipate that significant results could be obtained with more controlled conditions.

Consideration for Coronavirus Disease 2019 Drug Treatment in Patients with Epilepsy on Antiepileptic Drugs

Several medications are approved to treat coronavirus disease 2019 (COVID-19) in Korea including nirmatrelvir/ritonavir, remdesivir, and regdanvimab. There is potential drug-drug interaction between antiepileptic drugs (AEDs) and the medications used to treat COVID-19. Several AEDs such as phenytoin, carbamazepine, phenobarbital, and primidone are strong cytochrome P450 inducers and can inhibit the drugs used for COVID-19. Particularly, these drugs are contraindicated with nirmatrelvir/ritonavir (Paxlovid®). There is a weaker drug-drug interaction between the AEDs and remdesivir. No significant interaction has been reported between the AEDs and molnupiravir. Pharmacokinetic interactions of the AEDs are important in effective management of COVID-19 in patients with epilepsy.

Clinical Usefulness of Intraoperative Motor-Evoked Potential Monitoring during Temporal Lobe Epilepsy Surgery

BACKGROUND AND PURPOSE: We aimed to determine the effectiveness of intraoperative neurophysiological monitoring focused on the transcranial motor-evoked potential (MEP) in patients with medically refractory temporal lobe epilepsy (TLE). METHODS: We compared postoperative neurological deficits in patients who underwent TLE surgery with or without transcranial MEPs combined with somatosensory evoked potential (SSEP) monitoring between January 1995 and June 2018. Transcranial motor stimulation was performed using subdermal electrodes, and MEP responses were recorded in the four extremity muscles. A decrease of more than 50% in the MEP or the SSEP amplitudes compared with baseline was used as a warning criterion. RESULTS: In the TLE surgery group without MEP monitoring, postoperative permanent motor deficits newly developed in 7 of 613 patients. In contrast, no permanent motor deficit occurred in 279 patients who received transcranial MEP and SSEP monitoring. Ten patients who exhibited decreases of more than 50% in the MEP amplitude recovered completely, although two cases showed transient motor deficits that recovered within 3 months postoperatively. CONCLUSIONS: Intraoperative transcranial MEP monitoring during TLE surgery allowed the prompt detection and appropriate correction of injuries to the motor nervous system or ischemic stroke. Intraoperative transcranial MEP monitoring is a reliable modality for minimizing motor deficits in TLE surgery.

2018 KHRS guideline for the evaluation and management of syncope: Part 2

The general concept and initial approach to syncope patients has been mentioned in the general sections. This special sections have been described the characteristics, diagnosis, and treatment with patient education for the each syncope. It has been described in order of reflex syncope, orthostatic hypotension, postural orthostatic tachycardia syndrome (POTS), cardiac syncope, and unexplained syncope. Several clinical issues will have been dealt with in special issues. Neurological assessment is added when the patients were diagnosed with psychogenic pseudosyncope (PPS). Although many childhood syncope caused by reflex syncope, they are also presented as syncope caused by arrhythmic events in patients with congenital heart disease. In the elderly patients, syncope is because of not only a single cause of syncope but a combination of various conditions. In case of a syncope patient visiting the emergency department, a standardized systematic approach will be required to determine whether hospitalize the patient according to the risk of recurrence and the needs for the syncope management unit. We also mention recommendations on the limits of driving, exercising and social life style that are relevant to syncope in all patients. In this guideline, we reviewed the Korean published literatures and European/American guidelines on syncope. We, writing and publishing committee for evaluation and management guidelines of syncope in the Korean Society for Holter and Noninvasive Electrocardiography (KSHNE) under the Korean Heart Rhythm Society (KHRS) are very pleased to be able to publish this guideline. We also hope this guideline will be a good support to manage the syncope patients and a useful trigger for further research in Korea.

2018 KHRS guideline for the evaluation and management of syncope: Part 1

Syncope is a very common symptom that occurs in all age groups, especially in adolescents and elderly people. The cause of syncope is very diverse, and patients with syncope visit various medical departments such as general medicine, cardiology, neurology, and emergency medicine. If we do not perform appropriate diagnostic tests based on detailed history of syncope, we may have some difficulty to identify the cause of syncope. Failure to identify the cause of syncope can lead to physical trauma due to recurrence of syncope or may increase the risk of cardiovascular events in the future. However, there is no Korean guidelines for the diagnosis and treatment of syncope yet. Considering these circumstances in Korea, we prepared writing and publishing committee for evaluation and management guidelines of syncope in the Korean Society for Holter and Noninvasive Electrocardiology (KSHNE) under the Korean Heart Rhythm Society (KHRS). In this guideline, we reviewed the Korean published literatures and European / American guidelines on syncope.

Hematoma-Related Nonhabitual Seizures during Subdural Electrode Monitoring

No abstract available.

Erratum to: Asymmetric Gray Matter Volume Changes Associated with Epilepsy Duration and Seizure Frequency in Temporal-Lobe-Epilepsy Patients with Favorable Surgical Outcome

Jeong Sik Kim(a,b*), Dae Lim Koo(c*), Mi-Ri Kang(a), Min Jae Seong(a), Won Gu Lee(a), Eun Yeon Joo(a,b), Sung Tae Kim(d), Dae Won Seo(a,b), Seung Bong Hong(a,b)

Asymmetric Gray Matter Volume Changes Associated with Epilepsy Duration and Seizure Frequency in Temporal-Lobe-Epilepsy Patients with Favorable Surgical Outcome

BACKGROUND AND PURPOSE: This study aimed to estimate the changes in gray matter volume (GMV) and their hemispheric difference in patients with mesial temporal lobe epilepsy (MTLE) using a voxel-based morphometry (VBM) methodology, and to determine whether GMV changes are correlated with clinical features. METHODS: VBM analysis of brain MRI using statistical parametric mapping 8 (SPM8) was performed for 30 left MTLE (LMTLE) and 30 right MTLE (RMTLE) patients and 30 age- and sex-matched healthy controls. We also analyzed the correlations between GMV changes and clinical features of MTLE patients. RESULTS: In SPM8-based analyses, MTLE patients showed significant GMV reductions in the hippocampus ipsilateral to the epileptic focus, bilateral thalamus, and contralateral putamen in LMTLE patients. The GMV reductions were more extensive in the ipsilateral hippocampus, thalamus, caudate, putamen, uncus, insula, inferior temporal gyrus, middle occipital gyrus, cerebellum, and paracentral lobule in RMTLE patients. These patients also exhibited notable reductions of GMV in the contralateral hippocampus, thalamus, caudate, putamen, and inferior frontal gyrus. We observed that GMV reduction was positively correlated with several clinical features (epilepsy duration and seizure frequency in RMTLE, and history of febrile seizure in LMTLE) and negatively correlated with seizure onset age in both the RMTLE and LMTLE groups. CONCLUSIONS: Our study revealed GMV decreases in the hippocampus and extrahippocampal regions. Furthermore, the GMV reduction was more extensive in the RMTLE group than in the LMTLE group, since it included the contralateral hemisphere in the former. This difference in the GMV reduction patterns between LMTLE and RMTLE may be related to a longer epilepsy duration and higher seizure frequency in the latter.

Absence of HLA-B*1502 and HLA-A*3101 Alleles in 9 Korean Patients With Antiepileptic Drug-Induced Skin Rash: A Preliminary Study

There have been a number of studies about correlations between HLA genotypes in various ethnic groups and occurrence of various cutaneous adverse drug reactions, ranging in intensity from mild to severe, caused by antiepileptic drugs (AEDs). This is the first report analyzing the HLA genotypes of 9 Korean patients with skin rashes induced by various AEDs. The AEDs that induced skin rash were lamotrigine (n=3), carbamazepine (n=3), oxcarbazepine (n=1), phenobarbital (n=1), and phenytoin (n=1). None of the patients' HLA genotypes was either HLA-B*1502 or HLA-A*3101. Based on these series of cases, AED-induced skin rash can occur independently of HLA-B*1502 or HLA-A*3101 genotypes in the Korean patients.

Intraoperative Neurophysiologic Monitoring: Basic Principles and Recent Update

The recent developments of new devices and advances in anesthesiology have greatly improved the utility and accuracy of intraoperative neurophysiological monitoring (IOM). Herein, we review the basic principles of the electrophysiological methods employed under IOM in the operating room. These include motor evoked potentials, somatosensory evoked potentials, electroencephalography, electromyography, brainstem auditory evoked potentials, and visual evoked potentials. Most of these techniques have certain limitations and their utility is still being debated. In this review, we also discuss the optimal stimulation/recording method for each of these modalities during individual surgeries as well as the diverse criteria for alarm signs.

Eye Manifestations during the Syncopal Attack in Head-up Tilt Tests

PURPOSE: Recently syncope can be recored on video during head-up tilting table test (HUT). We assess the eye signs during the syncope to characterize eye signs of syncope.METHODS: We tried HUT based on the SMC protocol. The protocol includes video setting for eye observation. We included the subjects who had HUT at Samsung Medical Center, Seoul, Korea between October 2010 and June 2011. We investigated the video of the patients with positive results and syncope during HUT, especially focusing on the eye signs including eye opening and eyeball position. We analyzed the differences of hemodynamic parameters between eye-open and eye-close groups.RESULTS: Of 380 patients with positive HUT, 53 (13.9%) patients experienced syncope during the test. Among the syncope patients, 31 (58.5%) patients showed eye opening during the syncopal attack and 22 (41.5%) patients revealed eye closure. When the syncopal patient had eye open, the eyeball position was fixed in 22 patients. Upward deviation was most common position. Eye opening group showed significant decrease of systolic blood pressure and heart rate during syncopal attack compared with eye closing group (p=0.044, p=0.041).CONCLUSIONS: We concluded that eye opening state during syncopal attack can be the sign of more severe degree of cardiohemodynamic dysfunction.

Pharmacokinetics and Therapeutic Drug Monitoring of Newer Antiepileptic Drugs

Recently a newer generation of antiepileptic drugs has been developed and rational polytherapy has become popular in everyday clinical practice for epilepsy management. All of newer antiepileptic drugs (AED) have undergone extensive clinical studies, but the information on the relationship between concentrations and effects is scarce. The concentration can depend on the individual variation, but explain the efficacy and toxicity in individual patient management. We assess the potential value of measuring the level of newer AED, which is one of therapeutic drug monitoring (TDM). The value of TDM is discussed based on pharmacokinetic characteristics and drug interactions. Although routine TDM in general cannot be recommended, TDM of the newer AED may be of value in selected cases.

Clinical Performance Examination Utilizing Standardized Patients in Board Examination: Based on the Board Examination of Korean Neurological Association for Three Years / 한국의학교육

PURPOSE: Evaluation of clinical skills and attitude including development of dynamic patient-doctor relationship is important in board examination (BE). Korean Neurological Association (KNA) has introduced clinical performance examination (CPX) utilizing standardized patients (SP) to BE in 2007. In this study, the authors describe the 3-year experience of CPX in BE through 2009. METHODS: To implement CPX session in BE, KNA developed CPX workshop for BE attendees and members of grading committee. CPX sessions in BE consisted of two model scenarios mimicking neurological patients in clinical practice. The total score and itemized scores of CPX sessions were compared with other areas of BE, and scores from each year were also compared. RESULTS: Scores from CPX sessions were significantly correlated with BE step II. Among the itemized scores of CPX sessions, clinical items including history taking and physical examination were significantly correlated with scores from other areas of BE. However, scores from global assessment from SP were strongly associated with patient-doctor relationship, history taking, and patient education. CONCLUSION: Our experiences suggest that CPX utilizing SP is a useful tool to assess the clinical skills in BE. In order to produce clinically well qualified neurologists, more efforts should be made to develop cases and to improve assessment tools for CPX.

Eye Manifestations during the Syncopal Attack in Head-up Tilt Tests / 대한간질학회지

PURPOSE: Recently syncope can be recored on video during head-up tilting table test (HUT). We assess the eye signs during the syncope to characterize eye signs of syncope. METHODS: We tried HUT based on the SMC protocol. The protocol includes video setting for eye observation. We included the subjects who had HUT at Samsung Medical Center, Seoul, Korea between October 2010 and June 2011. We investigated the video of the patients with positive results and syncope during HUT, especially focusing on the eye signs including eye opening and eyeball position. We analyzed the differences of hemodynamic parameters between eye-open and eye-close groups. RESULTS: Of 380 patients with positive HUT, 53 (13.9%) patients experienced syncope during the test. Among the syncope patients, 31 (58.5%) patients showed eye opening during the syncopal attack and 22 (41.5%) patients revealed eye closure. When the syncopal patient had eye open, the eyeball position was fixed in 22 patients. Upward deviation was most common position. Eye opening group showed significant decrease of systolic blood pressure and heart rate during syncopal attack compared with eye closing group (p=0.044, p=0.041). CONCLUSIONS: We concluded that eye opening state during syncopal attack can be the sign of more severe degree of cardiohemodynamic dysfunction.

Pharmacokinetics and Therapeutic Drug Monitoring of Newer Antiepileptic Drugs / 대한간질학회지

Recently a newer generation of antiepileptic drugs has been developed and rational polytherapy has become popular in everyday clinical practice for epilepsy management. All of newer antiepileptic drugs (AED) have undergone extensive clinical studies, but the information on the relationship between concentrations and effects is scarce. The concentration can depend on the individual variation, but explain the efficacy and toxicity in individual patient management. We assess the potential value of measuring the level of newer AED, which is one of therapeutic drug monitoring (TDM). The value of TDM is discussed based on pharmacokinetic characteristics and drug interactions. Although routine TDM in general cannot be recommended, TDM of the newer AED may be of value in selected cases.

Quality of Life Assessment in Epilepsy Patients With Topiramate

BACKGROUND: Quality of life is important in epilepsy treatment. We evaluated the effect of topiramate on the quality of life in epilepsy patients and on the characteristics of the clinical features in patients with improved scores on the Quality of Life in Epilepsy Inventory-10 (QOLIE-10). METHODS: We included 631 adult epilepsy patients, who were followed for 16 weeks. We analyzed the effect of topiramate on QOLIE-10 score. The patients were classified into those with improved scores (IG) and those whose scores did not improve (NG). We compared each QOLIE-10 item and their clinical features between the IG and NG groups. RESULTS: All item scores, as well as the total score, improved after topiramate treatment. Comparison of the scores for each item showed that working and social factor differences of both the IG (>0.72) and NG groups (>0.80) were higher than for the other items. Age, gender, seizure onset, topiramate dose, adverse event, education level, employment, and migraine did not differ significantly between the IG and NG groups. The effect on seizure reduction was the most significant among the clinical features (P<0.001). The epilepsy syndrome, etiology, previous antiepileptic drugs (AEDs), and weight changes did not differ significantly between the two groups (P<0.05). CONCLUSIONS: Topiramate treatment can influence the quality of life in adult epilepsy patients, and especially work and social factors. Seizure reduction, epilepsy syndrome, etiology, previous AEDs, and weight change may be the most affected clinical factors in patients who have improved quality of life with topiramate.

Vertebrobasilar Insufficiency Caused by Lateral Head Tilting

Vertebrobasilar insufficiency (VBI) in rotatory vertebral artery occlusion syndrome can be caused by voluntary head turning in the yaw plane. We report a case of VBI for head tilting in the roll plane. Transcranial Doppler on left head tilting resulted in decreased blood flow in both vertebral arteries. A CT angiogram revealed that the stenotic left vertebral artery was very close to an enlarged thyroid gland, suggesting mechanical compression of the vertebral artery during head tilt.

Surgical Treatment of Intractable Epilepsy Originating from Central Region: A Case Report / 대한간질학회지

Localization of epileptogenic zone in central epilepsy is difficult because of extent and rapidity of ictal spread. Epilepsy surgery in central region is challenging because of the risk of sensorimotor deficitsrelated to excision of eloquent areas. We present a case of 27- year-old woman with a successful surgical treatment for intractable epilepsy from the central region.

Postoperative Electroclinical Features in Epilepsy Patients With Seizures After Anteromesial Temporal Resection

BACKGROUND: Anteromesial temporal resection (AMTR) is well established as effective in patients with intractable mesial temporal epilepsy. However, little electroclinical information is available relevant to poor surgical outcome after AMTR. We examined the postoperative electroclinical features based on postoperative MRI and video-EEG monitoring (VEM) in patients with poor surgical outcome. METHODS: We reviewed clinical features and postoperative VEM results in 20 patients with failure in AMTR. According to the postoperative electroclinical features, we classified them into mesial temporal (MT), bitemporal (BT), extramesial temporal (XMT), combined (C), and unclassified groups. The postoperative VEM results were compared among the groups. Surgical outcome was assessed in five patients who underwent reoperation. RESULTS: Patients comprised 6 MT, 2 BT, 6 XMT, 1 C, and 6 unclassified. Aura and automatism were more frequent in MT (50.0%, 83.3%) than in XMT (16.7%, 33.3%). Theta to delta rhythm, during the ictal onset and build-up period, was more frequent in MT (83.3%, 66.7%) than in XMT (33.3%, 33.3%). The ictal onset and build-up pattern of ictal EEG were most frequently localized to the frontotemporal region in MT (66.7%, 100.0%), while there was no predominantly localized region in XMT. The surgical outcome after reoperation was better in MT group than in XMT and C groups. CONCLUSIONS: Postoperative MRI and VEM are useful to assess the postoperative electroclinical features in failed AMTR. Reoperation of the residual mesiotemporal structures after confirming epileptogenic foci may have good surgical outcome.