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Background: Childhood epilepsy with centrotemporal spikes (CECTS) is the most common epilepsy syndrome in school-aged children. However, predictors for seizure frequency are yet to be clarified using the phase lag index (PLI) analyses. We investigated PLI of scalp electroencephalography data at onset to identify potential predictive markers for seizure times. Methods: We compared the PLIs of 13 patients with CECTS and 13 age- and sex-matched healthy controls. For the PLI analysis, we used resting-state electroencephalography data (excluding paroxysmal discharges), and analyzed the mean PLIs among all electrodes and between interest electrodes (C3, C4, P3, P4, T3, and T4) and other electrodes. Furthermore, we compared PLIs between CECTS and control data and analyzed the associations between PLIs and total seizure times in CECTS patients. Results: No differences were detected in clinical profiles or visual electroencephalography examinations between patients with CECTS and control participants. In patients with CECTS, the mean PLIs among all electrodes and toward interest electrodes were higher at the theta and alpha bands and lower at the delta and gamma bands than those in control participants. Additionally, the mean PLIs toward interest electrodes in the beta frequency band were negatively associated with seizure times (P = 0.02). Conclusion: The resting-state delta, theta, alpha, and gamma band PLIs might reflect an aberrant brain network in patients with CECTS. The resting-state PLI among the selected electrodes of interest in the beta frequency band may be a predictive marker of seizure times in patients with CECTS.

This study aimed to determine the correlation between outcomes following adrenocorticotrophic hormone (ACTH) therapy and measurements of relative power spectrum (rPS), weighted phase lag index (wPLI), and graph theoretical analysis on pretreatment electroencephalography (EEG) in infants with non-lesional infantile epileptic spasms syndrome (IESS).Twenty-eight patients with non-lesional IESS were enrolled. Outcomes were classified based on seizure recurrence following ACTH therapy: seizure-free (F, n = 21) and seizure-recurrence (R, n = 7) groups. The rPS, wPLI, clustering coefficient, and betweenness centrality were calculated on pretreatment EEG and were statistically analyzed to determine the correlation with outcomes following ACTH therapy.The rPS value was significantly higher in the delta frequency band in group R than in group F (p 0.001). The wPLI values were significantly higher in the delta, theta, and alpha frequency bands in group R than in group F (p = 0.007,0.001, and0.001, respectively). The clustering coefficient in the delta frequency band was significantly lower in group R than in group F (p 0.001).Our findings demonstrate the significant differences in power and functional connectivity between outcome groups.This study may contribute to an early prediction of ACTH therapy outcomes and thus help in the development of appropriate treatment strategies.

Background Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by biphasic seizures and white matter lesions with reduced diffusion, which are often accompanied by involuntary movements. The neurological outcomes of AESD vary from normal to mild or severe sequelae, including intellectual disability, paralysis, and epilepsy. The present study aimed to clarify the prognostic factors of AESD, including involuntary movements. Methods We enrolled 29 patients with AESD admitted to Tottori University Hospital from 1991 to 2020 and retrospectively analyzed their clinical data. Neurological outcomes were assessed by the Pediatric Cerebral Performance Category score and cerebral paralysis as neurological sequelae. Results Of the 29 patients, 12 had favorable outcomes and 17 had unfavorable outcomes. Univariate analysis revealed that the presence of underlying diseases, a decline in Glasgow Coma Scale (GCS) score 12–24 h after early seizures, and involuntary movements were associated with unfavorable outcomes. In multivariate analysis, a decline in GCS score and involuntary movements were associated with unfavorable outcomes. The sensitivities and specificities of underlying diseases, a decline of ≥ 3 points in GCS score 12–24 h after early seizures, and involuntary movements for unfavorable outcomes were 53% and 92%, 92% and 65%, and 59% and 92%, respectively. Conclusions The appearance of involuntary movements may be associated with unfavorable outcomes of AESD. The prognostic factors identified herein are comparable with previously known prognostic factors of consciousness disturbances after early seizures.

Background Incontinentia pigmenti (IP) is an X-linked neurocutaneous disorder that can present with cerebral arteriopathy during early infancy. However, no previous reports have demonstrated arteriopathic manifestations during postinfantile childhood in patients with IP. Patient description We describe a case of IP in a 2-year-old girl who developed encephalopathic manifestations associated with influenza A infection. She presented diffuse magnetic resonance imaging abnormalities involving the cortices, subcortical white matter, corpus callosum, basal ganglia, and thalami, resembling the findings in early infantile cases reported in the previous literatures. Magnetic resonance angiography demonstrated attenuation of the cerebral arteries. Proinflammatory cytokines and chemokines were upregulated in the cerebrospinal fluid. Left hemiplegia remained following the remission of the arteriopathic manifestations. Genetic analyses revealed a novel type of mutation in the IKBKG gene. Conclusion Our findings indicate that patients with IP can develop destructive cerebral arteriopathy even after early infancy. The similarities in magnetic resonance imaging abnormalities between our patient and the previously reported infantile patients may be explained by the underlying immunologic pathophysiology of IP.

Background: To clarify the differences in diaphragm thickness between male and female participants in healthy young adults using ultrasonography and to investigate the relationship between diaphragm thickness and respiratory pressure.Methods: Twenty-nine healthy individuals (16 females and 13 males) participated in the study. Diaphragm thickness was measured at total lung capacity (TLC) and at functional residual capacity (FRC) in each participant. We measured the diaphragm thickness using a method for mean intima media thickness. Moreover, change ratio of diaphragm thickness was calculated with the diaphragm thickness at TLC and FRC.Results: Mean diaphragm thicknesses at FRC in males were significantly narrower than those in females (pConclusions: The change ratio might be an alternative to evaluate respiratory muscle strength in young adult males. The diaphragm thickness values and the change ratio of diaphragm thickness in healthy young adults, evaluated by B-mode sonography, were varied based on sex.

Background Patients with MECP2 duplication syndrome present with distinct facial anomalies and clinical features such as global developmental delay, recurrent respiratory infections, and epileptic seizures. Approximately half of all patients develop epileptic seizures which are refractory in most cases despite active medical management. Furthermore, no previous reports have discussed the efficacy of surgical treatment for seizures in patients with MECP2 duplication syndrome. Case report In the present report, we describe a case of MECP2 duplication syndrome in a 15-year-old boy who developed epileptic seizures following influenza-associated acute encephalitis. His frequent epileptic spasms, tonic, atonic, and partial seizures were refractory to multiple antiepileptic medications. Electroencephalography revealed continuous diffuse epileptic discharge, resulting in regression. A total corpus callosotomy (CC) was performed at the age of 14 years and 7 months. His seizures markedly decreased following CC, although he continued to experience brief partial seizures approximately once per month. Post-operative examination revealed that his epileptic discharges had disappeared, and that his developmental state had returned to pre-encephalopathy levels. Conclusion Our findings indicate that CC may represent a valuable surgical option for children with medically refractory generalized seizures following acute encephalopathy, irrespective of genetic disorders such as MECP2 duplication syndrome.

Objectives Long‐term adrenocorticotropic therapy (LT‐ACTH), which consisted of 2‐4 weeks of daily injections of adrenocorticotropic hormone (ACTH) and subsequent months of weekly injections, was tried for relapsed West syndrome (WS) or other intractable epilepsies in small case reports. Our aim was to explore the efficacy of LT‐ACTH for preventing WS relapse, as well as the prevalence of its adverse events. Methods This is a retrospective, nationwide, multicenter case series of patients with WS who underwent LT‐ACTH. Clinical information of the patients and protocol of LT‐ACTH were collected from participating institutes in this study. We defined clinical response to ACTH as achievement of hypsarrhythmia and epileptic spasms resolution. Patients who responded to daily ACTH injections were identified and assessed whether they experienced WS relapse during/after the weekly ACTH injection period. The outcome was measured by the nonrelapse rate at 24 months after daily ACTH injections using the Kaplan‐Meier method. Results Clinical information of 16 children with WS was analyzed. The median age at LT‐ACTH initiation was 14.5 months (range: 7‐68 months). Thirteen (81%) patients had previously undergone conventional ACTH treatment. The LT‐ACTH regimens comprised a median of 16 days of daily injections (range: 11‐28 days) and 10 months of weekly injections (range: 3‐22 months). Seven patients experienced WS relapse during/after subsequent weekly ACTH period, and the nonrelapse rate at 24 months after daily injections was estimated at 60.6% (95% confidence interval: 32.3%‐80.0%). Height stagnation, hypertension, and irritability were observed; lethal adverse events were not reported. Significance Our study firstly explored the efficacy of LT‐ACTH for preventing WS relapse. LT‐ACTH might be a treatment option for patients with relapsed or intractable WS; however, we note that our study is limited by its small sample size and the lack of an appropriate control group.

When the results of electroencephalography (EEG), magnetic resonance imaging (MRI), and seizure semiology are discordant or no structural lesion is evident on MRI, single-photon emission computed tomography (SPECT) and positron emission tomography (PET) are important examinations for lateralization or localization of epileptic regions. We hypothesized that the concordance between interictal 2-[18F]fluoro-2-deoxy-D-glucose (18FDG)-PET and iomazenil (IMZ)-SPECT could suggest the epileptogenic lobe in patients with non-lesional findings on MRI. Fifty-nine patients (31 females, 28 males; mean age, 29 years; median age, 27 years; range, 7–56 years) underwent subdural electrode implantation followed by focus resection. All patients underwent 18FDG-PET, IMZ-SPECT, and focus resection surgery. Follow-up was continued for ≥ 2 years. We evaluated surgical outcomes as seizure-free or not and analyzed correlations between outcomes and concordances of low-uptake lobes on PET, SPECT, or both PET and SPECT to the resection lobes. We used uni- and multivariate logistic regression analyses. In univariate analyses, all three concordances correlated significantly with seizure-free outcomes (PET, p = 0.017; SPECT, p = 0.030; both PET and SPECT, p = 0.006). In multivariate analysis, concordance between resection and low-uptake lobes in both PET and SPECT correlated significantly with seizure-free outcomes (p = 0.004). The odds ratio was 6.0. Concordance between interictal 18FDG-PET and IMZ-SPECT suggested that the epileptogenic lobe is six times better than each examination alone among patients with non-lesional findings on MRI. IMZ-SPECT and 18FDG-PET are complementary examinations in the assessment of localization-related epilepsy.

Background Patients with infection-related acute encephalitis sometimes develop epilepsy in the chronic phase of the disease. Patients with postencephalitic epilepsy usually develop partial seizures due to the lesions generated by the encephalitis. We report a case who developed late-onset epileptic spasms after acute cerebral and cerebellar encephalitis. Case report: A 5-year-old girl showed severe tremor, gait ataxia, partial or generalized tonic-clonic seizures, hyperactivity, and panic attacks after a mild enterocolitis. Her cerebellar symptoms disappeared until 3 months after onset, and her seizures were controlled with carbamazepine. However, the seizures reappeared as epileptic spasms 5 months after onset. The anti-NMDA-type glutamate receptor antibody concentration was significantly elevated in her cerebrospinal fluid at 8 days, 10 months, and 15 months after onset. The spasms were resistant to multiple antiepileptic drugs. High-dose methylprednisolone and high-dose immunoglobulin therapies did not show any benefits. Oral pranlukast hydrate was started 17 months after onset. After 3 weeks of the medication, her seizures disappeared, and her behavior also dramatically improved. Conclusion We presented a rare case of post-encephalitic epilepsy that manifested as epileptic spasms. Pranlukast significantly improved her seizures.

Background Vagus nerve stimulation (VNS) leads to palliation of refractory seizures. Epileptic spasms (ES) and tonic spasms (TS) appear in children with West syndrome and symptomatic generalized epilepsy. Both types of spasms are often characterized by truncal muscular contractions and ictal electroencephalography (EEG) findings comprising the contiguous phases: phase 1) 15–20 Hz, spindle-like fast activity (occur in 70%), 2) diffuse polyphasic δ/θ waves (100%), and 3) electrodecremental activity (70%). Here, we examined the effect of VNS on these spasms that are uniformly associated with the EEG and electromyogram changes. Methods A consecutive series of 32 patients satisfied the inclusion criteria consisting of 1) medically refractory epilepsy, 2) VNS implantation between 2010 and 2015, 3) implantation of VNS before the age of 20 years, and 4) follow-up >2 years. From this cohort, 16 patients had spasms (ES/TS group), whereas the remaining 16 had partial seizures with or without secondary generalization (PS/SG group). We compared seizure outcomes between the two groups, and also determined the factors predicting these outcomes within the ES/TS group. Results The outcomes after 2 years of implantation, defined using the McHugh classification, were as follows: II (for 2 patients), III (5), and V (9) in the ES/TS group; and I (3 patients), II (6), III (2), IV (1), and V (4) in the PS/SG group. The ES/TS group had significantly worse outcomes than the PS/SG group (p = 0.024, Mann-Whitney U test). Multivariate ordinal logistic regression analysis revealed that shorter mean durations of ictal events were associated with better seizure outcomes following VNS implantation (p = 0.007). Significance Only 13% of the patients in the ES/TS group had seizure reductions of greater than 50%. VNS was less effective for the treatment of patients with ES/TS than for those with PS/SG and those described in previous studies.

BCL11A encodes a zinc finger protein that is highly expressed in hematopoietic tissues and the brain, and that is known to function as a transcriptional repressor of fetal hemoglobin (HbF). Recently, de novo variants in BCL11A have been reported in individuals with intellectual disability syndrome without epilepsy. In this study, we performed whole-exome sequencing of 302 patients with epileptic encephalopathies (EEs), and identified 2 novel BCL11A variants, c.577delC (p.His193Metfs*3) and c.2351A>C (p.Lys784Thr). Both the patients shared major physical features characteristic of BCL11A-related intellectual disability syndrome, suggesting that characteristic physical features and the persistence of HbF should lead clinicians to suspect EEs caused by BCL11A pathogenic variants. Patient 1, with a frameshift variant, presented with Lennox-Gastaut syndrome, which expands the phenotypic spectrum of BCL11A haploinsufficiency. Patient 2, with a p.Lys784Thr variant, presented with West syndrome followed by drug-resistant focal seizures and more severe developmental disability. These 2 newly described patients contribute to delineating the associated, yet uncertain phenotypic characteristics of BCL11A disease-causing variants.

Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a rare condition. The seizure control of this encephalopathy is poor. The purpose of this report is to evaluate the utility of vagus nerve stimulation (VNS) in a patient with AERRPS. A 14-year-old boy presented with fever and headache. He developed epileptic facial twitching and eyelid fluttering. Various types of antiepileptic drugs did not control his seizures. He underwent VNS at the age of 21.After 5 years of VNS therapy, his seizure frequency decreased from daily to monthly.For patients with AERRPS, VNS may be a treatment option. Keywords: Post-encephalitic epilepsy, Vagus nerve stimulation therapy, Acute encephalitis with refractory, repetitive partial seizures (AERRPS)

Stereoelectroencephalography (SEEG) is an invasive surgical procedure used to identify epileptogenic zones. The combination of both subdural grids and depth electrodes (DEs) is currently used for invasive intracranial monitoring in many epilepsy centers. To perform DE implantation, some centers use frame-based stereotactic techniques and others use stereotactic robotic techniques. However, not all epilepsy centers have access to these tools. We hypothesized that DE implantation using a neuronavigation system can be utilized for subsequent epilepsy surgery. Between April 2016 and April 2017, we performed invasive monitoring for 26 patients. Among these, 17 patients (8 females, 9 males; mean age, 21.2 years; range, 3-51 years) underwent DE implantation. We divided patients into three groups: Group 1 (7 patients), a free-hand implantation group; Group 2 (7 patients), a frameless stereotactic implantation group; and Group 3 (3 patients), a computed tomography (CT)-guided auto image registration system with the stereotactic implantation group. Group 3 showed the closest distance from planned target to DE tip, followed by Group 2. Fourteen of the 17 patients underwent subsequent epilepsy surgery referring to the results of DE studies. DE placement using a neuronavigation system without stereotactic robotic equipment or frame-based stereotactic techniques can be utilized for subsequent epilepsy surgery.

We aimed to analyse the ictal electrographic changes on scalp electroencephalography (EEG), focusing on high-voltage slow waves (HVSs) in children with epileptic spasms (ES) and tonic spasms (TS) and then identified factors associated with corpus callosotomy (CC) outcomes. We enrolled 17 patients with ES/TS who underwent CC before 20 years of age. Post-CC Engel’s classification was as follows: I in 7 patients, II in 2, III in 4, and IV in 4. Welch’s t-test was used to analyse the correlation between ictal HVSs and CC outcomes based on the following three symmetrical indices: (1) negative peak delay: interhemispheric delay between negative peaks; (2) amplitude ratio: interhemispheric ratio of amplitude values for the highest positive peaks; and (3) duration ratio: interhemispheric ratio of slow wave duration. Ages at CC ranged from 17–237 months. Four to 15 ictal EEGs were analysed for each patient. The negative peak delay, amplitude ratio and duration ratio ranged from 0–530 ms, 1.00–7.40 and 1.00–2.74, respectively. The negative peak delay, amplitude ratio and duration ratio were significantly higher in the seizure residual group (p = 0.017

Objective We aimed to clarify the association between magnetic resonance imaging (MRI)-lesion patterns, including cortices and white matters, and the development, occurrence, and intractableness of West syndrome in patients with tuberous sclerosis complex (TSC), using visual analysis. Methods We collected data for 44 patients with TSC who had undergone brain MRI and developmental evaluation after the ages of 2 and 3 years, respectively. Fluid-attenuated inversion recovery (FLAIR) and T1-weighted images were used to analyze the number of cyst-like tubers, the number of cyst-like subcortical lesions, and the presence of diffuse lesions involving the cortices and white matter. Results Developmental delays were observed in 28 patients. Nineteen patients had a history of West syndrome. Cyst-like tubers (range: 1–10), cyst-like subcortical lesions (range: 1–4), and diffuse lesions (range: 1–6 areas) were observed in 15, 9, and 14 patients, respectively. In the univariate analyses, all MRI findings were associated with development and/or history of West syndrome. However, in the multivariate analyses, only the diffuse lesion was associated with severe development (p = 0.003) and history of West syndrome (p = 0.012). In the subanalysis of patients with West syndrome, the diffuse lesions were also associated with pharmacological intractableness. Patients with diffuse lesions had a history of West syndrome with sensitivity of 68% and specificity of 96%. Patients with two or more areas of diffuse lesions had history of pharmacologically intractable West syndrome with sensitivity of 89% and specificity of 91%. Conclusions Diffuse lesions may help to predict the poor neurological outcomes in patients with TSC.

Background This retrospective study is designed to determine whether the thickness of the corpus callosum can predict corpus callosotomy outcome in pediatric patients with epileptic or tonic spasms. Methods We retrospectively studied 25 patients (18 boys) with intractable childhood-onset epileptic or tonic spasms who underwent corpus callosotomy between March 2008 and January 2017. Seizure outcomes were classified as favorable (class I and II of Engel's outcome classification) or unfavorable (class III and IV of Engel's outcome classification) at 12 months postoperatively. We measured the corpus callosum area on the midline and maximum cerebral area on the para-midline in sagittal magnetic resonance images just before surgery. We statistically analyzed the associations between surgical outcomes and corpus callosum area, corpus callosum area/maximum cerebral area (corpus callosum/cerebrum ratio), or age at magnetic resonance imaging just before surgery, using univariate and multivariate logistic regression analyses. Results Age at surgery ranged from six to 237 months (mean: 119). Main seizure types were epileptic spasms in 17 patients and tonic spasms in eight. Favorable outcomes occurred in 10 (40%) patients and unfavorable outcomes in 15 (60%). Both corpus callosum area and corpus callosum/cerebrum ratio did not show significant associations with the outcomes in the univariate and multivariate analyses. The 95% confidence intervals of corpus callosum/cerebrum ratio strongly overlapped between the favorable and unfavorable outcome groups. Conclusions Our data failed to support that corpus callosum thickness on the sagittal image is associated with corpus callosotomy outcomes in pediatric patients with epileptic spasms or tonic spasms.

Tuberous sclerosis complex is a genetic systematic disorder characterized by hamartomas in multiple organs. Cortical tubers, the hamartomas in the cerebrum, cause multifocal refractory seizures. In certain cases, epileptic foci potentially involve language areas, and hence, extra- and intraoperative cortical mapping can help identify anterior and posterior areas, thus avoiding postsurgical language impairment. We report on a 21-year-old female with tuberous sclerosis complex experiencing refractory partial seizures due to two epileptic foci in the left hemisphere overlapping anterior and posterior language areas. To completely evaluate both language areas, we performed stepwise resections beginning from the anterior to the posterior epileptic focus. Although the patient presented with expressive aphasia following anterior resection, it was possible to conduct language tests during every resection. Postoperatively, she presented with expressive aphasia, comprehension deficits, left-right disorientations, and arithmetic deficits. The language dysfunctions almost disappeared at 5 weeks after the surgery, and were completely resolved at 6 months after surgery. At postoperative 9 months, she was free from seizures.

When the results of electroencephalography (EEG), magnetic resonance imaging (MRI), and seizure semiology are discordant or no structural lesion is evident on MRI, single-photon emission computed tomography (SPECT) and positron emission tomography (PET) are important examinations for lateralization or localization of epileptic regions. We hypothesized that the concordance between interictal 2-[Fifty-nine patients (31 females, 28 males; mean age, 29 years; median age, 27 years; range, 7-56 years) underwent subdural electrode implantation followed by focus resection. All patients underwentIn univariate analyses, all three concordances correlated significantly with seizure-free outcomes (PET, p = 0.017; SPECT, p = 0.030; both PET and SPECT, p = 0.006). In multivariate analysis, concordance between resection and low-uptake lobes in both PET and SPECT correlated significantly with seizure-free outcomes (p = 0.004). The odds ratio was 6.0.Concordance between interictal

Purpose Polymicrogyria, a malformation of the cerebral cortex, frequently causes epilepsy. Diffuse bilateral polymicrogyria (DBP) is related to poor epilepsy prognosis, but most patients with DBP are not good candidates for resective epilepsy surgery and effectiveness of corpus callosotomy (CC), a palliative surgery, for patients without resective epileptogenic cortices, has not been established in DBP. Because CC might be effective against DBP-related epilepsy, we conducted total CC in three pediatric DBP cases. Methods Case 1. A girl developed epilepsy at 3 months of age, with focal versive seizures and epileptic spasms. The electroencephalogram (EEG) showed a suppression-burst pattern. Total CC was performed at 6 months of age. Case 2. A female infant developed epilepsy on the day of birth, exhibiting epileptic spasms, generalized tonic-clonic seizures, and eye-deviating seizures. She had a history of clusters of tonic seizures. Total CC was performed at 1 year and 2 months of age. After CC, the epileptic focus of the tonic seizures was identified; a secondary resective surgery was conducted. Case 3. A girl developed multiple types of seizures at 3 years of age. Frequent atypical absence status was refractory to antiepileptic drugs. Total CC was conducted at 8 years of age. Results Case 1: Frequencies of both seizure types decreased. The background EEG changed to continuous high-voltage slow waves. Case 2: Clusters of tonic seizures were well-controlled. Case 3: Atypical absence seizures completely disappeared. Conclusion CC could be effective for patients with DBP, whose habitual seizures include epileptic spasms and absence seizures.

Ictal scalp EEG patterns have been reported to vary across onset regions. We assessed the sequential EEG changes during seizure events on scalp EEG, and tested our hypothesis that patients with focal seizures arising from the cortex facing the interhemispheric fissure (IHF cortex) would be specifically characterized by 3 phase-EEG patterns (3Ph-EEG).Patient inclusion criteria were: 1) focal epilepsy and 2) ictal onset records on scalp and intracranial video-EEG. Patients were classified into one of the three groups: the IHF group, mesial temporal (MT) group, or lateral convexity (LC) group, based on the localization of the ictal onset zone (IOZ) on intracranial video-EEG. We defined 3Ph-EEG on ictal scalp EEG as follows: phase 1, brief spike/fast wave burst; phase 2, diffuse attenuation; and phase 3, focal rhythmic activity with evolution. We determined if the occurrence ratios of 3Ph-EEG and each of the three phases differed between the groups (IHF and others).We studied 36 patients aged 8-59 years (mean, 30 years). Ten patients were classified as IHF, 16 as MT, and 10 as LC group from 303 ictal events on intracranial EEG. 193 seizures on scalp EEG consisted of 79 seizures in IHF; 58 in MT; 56 in LC group. Sixty-nine seizures (92%) in nine patients (90%) in IHF group showed 3Ph-EEG, whereas none of the seizures in MT and LC groups showed the 3Ph-EEG. Multivariate logistic regression analysis suggested that the presence of 3Ph-EEG (p 0.01) and phase 2 (p = 0.03) components were predictive of IHF scalp EEG onset.The 3Ph-EEG may be observed preferentially in patients with focal seizures from IHF cortex. Observation of the ictal phases on scalp EEG may warrant consideration of intracranial EEG sampling for the presurgical evaluation.

Introduction Cerebral hemispherotomy is a surgical method with a high rate of seizure reduction in patients with intractable epilepsy. However, there is a probability of postoperative motor deficits. The objective of this study was to investigate whether the Wada test can help predict motor function outcomes after hemispherotomy and, therefore, may be useful in decision-making and patient selection. Patients and method A total of 13 patients with hemispherical intractable epilepsy underwent hemispherical disconnection surgeries. Six of them underwent the Wada test to evaluate motor function and language function followed by peri-insula hemispherotomy. The patients’ age ranged from 11 to 45 years (mean 27 years). Results Three of six patients had reduced dexterity on the Wada test. The finger motor function in the other patients did not change on the Wada test. Postoperatively, all patients who had decreased fine motor movement on the Wada test showed postoperative clumsiness of their hands and fingers. Conclusions The Wada test might predict postoperative fine finger motor deficit after hemispherotomy. This study showed that gross motor function was compensated in the ipsilateral hemisphere, whereas fine finger motor movement function remained in the contralateral frontal cortex.

Introduction The perfect match between pre-surgical estimated seizure onset zone and subdural electrodes (SEs) position is requested for epilepsy surgery. However, post-surgical neuro images sometimes disappoint physicians due to SEs malposition. To place SEs, we used to use intraoperative photograph and fluoroscopy. With this procedure, we just recognized approximate location of SEs during surgery. The purpose of the study is to perform precise SEs location intraoperatively using intra operative computer tomography (CT) and image-guidance. Patients and methods There were 12 patients with intractable epilepsy. The age ranged from 5 years old to 42 years old. They underwent SEs placement. The patients intraoperatively underwent brain CT. Their CT images were automatically fused onto their 3D magnetic resonance image (MRI) brain image at the monitor of the image-guidance. In case of malposition or under coverage, neurosurgeons revised or add SEs along with advice of epileptologists and neurophysiologists. Result Patients underwent intraoperative CT scans once to three times. Epileptologists, neurophysiologists and surgeons could intraoperatively recognize the relationship between the SEs and the 3D MRI brain surface. We could intraoperatively discuss the localization of SEs and correct the position or add more SEs to cover estimated seizure onset zone along with real-time visualized image. We could avoid postoperative malposition of the SEs, so there were no another SEs operation for revision. Conclusion The intraoperative real-time visualization of SEs on 3D brain surface image helped us to perform accurate electrodes placement and could avoid the electrode malposition.

Doose syndrome (epilepsy with myoclonic-atonic seizures) is an epilepsy syndrome with an incidence of approximately 1-2% of childhood-onset epilepsies. Although this syndrome is associated with multiple types of generalized seizures, the diagnosis is based on the presence of myoclonic-atonic seizures. Eighteen percent of patients have refractory seizures and intellectual disabilities. There have, however, been a few reports on the efficacy of surgical treatment for Doose syndrome. We describe a case of Doose syndrome in a 10-year-old boy. He developed generalized tonic-clonic seizures at 3years 8months of age and subsequently developed myoclonic-atonic, myoclonic, and tonic seizures. The frequent myoclonic seizures were refractory to multiple antiepileptic medications. His cognitive development was moderately delayed. Anterior four fifths corpus callosotomy was performed at 8years 5months of age. His seizures, especially myoclonic seizures, were markedly reduced. He was given vagus nerve stimulation therapy at 9years and 1month of age, which led to complete resolution of the myoclonic seizures. Corpus callosotomy can be a good treatment strategy in patients with Doose syndrome with medically refractory generalized seizures.

Wolf–Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

PurposeTo perform an epilepsy surgery on a patient with drug resistant epilepsy secondary to bilateral brain malformation. The patient was a 2-year 9-month-old boy who had congenital bilateral multiple abnormalities. He developed a complex partial seizure at 9 months old. Based on the presurgical evaluations, he underwent a right hemispherotomy.ResultsBrain MRI revealed congenital bilateral polymicrogyria, right schizencephaly, and corpus callosum agenesis. The abnormality was noted to be more severe in the right hemisphere. Diffusion tensor image tractography clearly detected a left pyramidal tract. On the contrary, the right hemisphere did not show a clear pyramidal tract. An interictal EEG showed epileptiform discharges over both hemispheres with right frontotemporal area dominancy. The long-term video-EEG recorded his habitual seizures arising from the right frontal area. Right hemispherotomy led to freedom from seizures and a reduced need for anti-epilepsy drugs.ConclusionsWe report a patient with bilateral MRI and EEG abnormalities with clear DTI laterality who achieved seizure freedom with epilepsy surgery.

Allogeneic stem cell transplantation is a curative treatment for severe congenital neutropenia (SCN). However, a standard conditioning regimen and donor source have not been established. We report 3 consecutive cases of SCN who were successfully treated by cord blood transplantation (CBT) with reduced-intensity conditioning consisting of fludarabine, melphalan, and low-dose total body irradiation. All cases achieved complete donor chimerism without severe infectious complications and have maintained normal neutrophil counts for between 3 and 9 years after CBT. These results suggest that CBT with reduced-intensity conditioning can be an alternative therapy for SCN when human leukocyte antigen-matched bone marrow donor is unavailable.

Trans- and cis-dendrochrysanines (1 and 2), isolated from the stems of Dendrobium chrysanthum Wall., were first synthesized from L-proline. The absolute configuration of 1 and 2 was established to be S by the syntheses.