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Purpose Forced normalization (FN) indicates psychotic episodes associated with seizure remission and disappearance of epileptiform activity on EEG. FN is likely to occur when frequent seizures are abruptly terminated by anti-epileptic drugs (AEDs) or epilepsy surgery. Methods We describe an atypical case of a patient with FN induced by lacosamide (LCM). Results A 23-year-old female patient with Lennox-Gastaut syndrome (LGS) was administered AEDs for LGS and hospitalised with weight loss and abnormal behaviour. Her condition fulfilled the FN criteria, which was considered to be induced by LCM. After a reduction in LCM dose, her abnormal behaviour and appetite improved. During LCM use, the patient developed no seizures, and the high amplitude diffuse sharp and slow wave complexes that were frequently observed before LCM disappeared on EEG. The LCM dose was tapered to 150 mg per day, and she became calmer with socially appropriate behaviours, although a few mild focal seizures relapsed. Conclusion LCM was effective for treating LGS in this patient and induced FN. Initially, it was difficult to recognise FN in cases of psychiatric disorders, especially in patients with intellectual disability. Patients with FN induced by LCM are rare, and only four patients have been previously reported who were treated by antipsychotic drug for psychosis.

Niemann–Pick disease type C1 (NPC1) is a fatal congenital neurodegenerative disorder caused by mutations in the NPC1 gene, which is involved in cholesterol transport in lysosomes. Broad clinical manifestations of NPC1 include liver failure, pulmonary disorder, neurological deficits, and psychiatric symptoms. The main cause of death in NPC1 patients involves central nervous system (CNS) dysfunction; there is no essential treatment. We generated a tyrosine-mutant adeno-associated virus (AAV) 9/3 vector that expresses human NPC1 under a cytomegalovirus (CMV) promoter (AAV-CMV-hNPC1) and injected it into the left lateral ventricle (5 μL) and cisterna magna (10 μL) of Npc1 homo-knockout (Npc1−/−) mice. Each mouse received total 1.35 × 1011 vector genome on days 4 or 5 of life. AAV-treated Npc1−/− mice (n = 11) had an average survival of >28 weeks, while all saline-treated Npc1−/− mice (n = 11) and untreated Npc1−/− mice (n = 6) died within 16 weeks. Saline-treated and untreated Npc1−/− mice lost body weight from 7 weeks until death. However, the average body weight of AAV-treated Npc1−/− mice increased until 15 weeks. AAV-treated Npc1−/− mice also showed a significant improvement in the rotarod test performance. A pathological analysis at 11 weeks showed that cerebellar Purkinje cells were preserved in AAV-treated Npc1−/− mice. In contrast, untreated Npc1−/− mice showed an almost total loss of cerebellar Purkinje cells. Combined injection into both the lateral ventricle and cisterna magna achieved broader delivery of the vector to the CNS, leading to better outcomes than noted in previous reports, with injection into the lateral ventricles or veins alone. In AAV-treated Npc1−/− mice, vector genome DNA was detected widely in the CNS and liver. Human NPC1 RNA was detected in the brain, liver, lung, and heart. Accumulated unesterified cholesterol in the liver was reduced in the AAV-treated Npc1−/− mice. Our results suggest the feasibility of gene therapy for patients with NPC1.

Twin-to-twin transfusion syndrome (TTTS) is a severe gestational complication due to an imbalance of blood flow between monochorionic diamniotic twins. TTTS can cause brain damage, most commonly ischemic lesions, but there are only a few reports of cortical dysplasia. We report the case of a TTTS donor twin with diffuse cortical dysplasia. Fetoscopic laser photocoagulation was performed at 18 weeks of gestation, and no abnormalities were noted in the subsequent course of the pregnancy; however, infantile spasms developed at 4 months after birth, and brain magnetic resonance imaging revealed diffuse cortical dysplasia. It was bilateral and widespread. A genetic abnormality was suspected, but no pathogenic mutation was detected. Hypoperfusion may have altered the expression of guidance factors, resulting in abnormal cortical formation.

Introduction Metachromatic leukodystrophy (MLD) refers to leukodystrophy caused by the accumulation of sulfatide from arylsulfatase A (ARSA) gene mutations. Sulfatide also accumulates in various organs, including the peripheral nerves, kidney, and gallbladder. Proliferative changes in the gallbladder have been reported in several patients, while gallbladder cancer is reported in only two adult MLD cases. We report what is likely the first pediatric case of MLD with gallbladder cancer. Case report The patient was a 5-year-old girl diagnosed with MLD using head magnetic resonance imaging and detecting a homozygous mutation of c.302G>A (p.Gly101Asp) in ARSA. Abdominal bloating was observed at the age of 4 years; CT revealed a giant tumor in the gallbladder and massive ascites. Cholecystectomy was performed and pathological examination revealed adenocarcinoma. Measurement of serum sulfatide revealed increased levels compared to the average healthy range. Discussion Rapidly increased ascites and large polyps which are reported as risk factors for cancer were characteristic in our MLD case. When such lesions are detected, they should be removed immediately because of the possibility of cancer, even in a pediatric patient.

Several patients with beta-propeller protein-associated neurodegeneration (BPAN)/static encephalopathy with neurodegeneration in adulthood have been reported to present Rett syndrome (RTT)-like features. This report presents an individual with BPAN showing clinical features of RTT. Psychomotor delay and epilepsy onset were noted at 1 year, and regression began at 4 years. Screening of the methyl-CpG binding protein 2 (MECP2) did not show variants. At 22 years, basal ganglia iron deposits were found on magnetic resonance imaging (MRI), and the WD-domain repeat 45 gene (WDR45) variant was identified. Review of the literature showed that BPAN with RTT-like features is associated with more epileptic seizures and less deceleration of head growth, breathing irregularities, and cold extremities than classic RTT with MECP2 variants. These clinical presentations may provide clues for differentiating between these two disorders. However, both WDR45 and MECP2 should be screened in patients presenting a clinical picture of RTT without specific MRI findings of BPAN.

Objective The early diagnosis of beta-propeller protein-associated neurodegeneration (BPAN) before distinct brain magnetic resonance imaging (MRI) findings of iron deposition occur remains challenging. This study examined whether children with BPAN have characteristic high-amplitude (>50 μV) fast activity (HAFA) on electroencephalography (EEG). Methods We conducted a retrospective analysis of EEG performed during childhood in five patients with BPAN. We also examined 143 EEGs from 59 patients with different etiologies, including epilepsy (n = 33), acute encephalopathy (n = 6), neurodevelopmental disorders (n = 5), non-epileptic events (n = 4), and others (n = 11). Trained electroencephalographers reviewed all of the EEGs. When excessive fast activity was observed, the amplitude, frequency, and locality were assessed. Results All five patients with BPAN underwent initial EEGs at 12–21 months old, and diffuse continuous HAFA (range 20–50 Hz) was observed on both awake and sleep EEGs. In the awake records, there was no clear posterior dominant rhythm in 4 of the 5 patients. Although 28% of the 143 EEGs had continuous excessive fast activity, mainly in the sleep records, only two (1.4%) exhibited HAFA when asleep, and their awake EEGs had clear posterior dominant rhythm. Conclusions The EEGs of children with BPAN showed diffuse HAFA continuously when both awake and asleep, which is uncommon in children with other etiologies. Significance This study provides an important clue for the early diagnosis of BPAN.

Background A report presenting five heterozygous de novo variants in VAMP2 in unrelated individuals with a neurodevelopmental disorder characterized by axial hypotonia, intellectual disability, and autistic features was first published in April 4, 2019. Case report We report the case of a male child with VAMP2 variant who was delivered at 38 weeks and 4 days without neonatal asphyxia. At 4 months of age he showed hypotonia and no visual pursuit and fixation. He presented with infantile spasms at 6 months, and electroencephalography (EEG) showed hypsarrhythmia. His infantile spasms completely disappeared by adrenocorticotropic hormone therapy, but his EEG findings continued to show high voltage slow-waves with multi-focal spikes. At 2 years of age he was non-verbal, had an absence of purposeful hand movements, and no visual fixation. He had somnolence tendency in the daytime. Biochemical and extensive genetic examinations were unrevealed. Magnetic resonance imaging showed slight brain atrophy. At 2 years and 7 months of age, he suffered from myoclonic seizures of the eyelid and tongue, which propagated to unilateral fingers, and sometimes to the bilateral legs. At 8 years of age hyperkinetic movement occurred. At age 13, whole-exome sequence identified a heterozygous missense variant, NM_014232.2:c.199G>C,[p.(Ala67Pro)] in exon 3 of VAMP2 which was a de novo non-synonymous variant. Conclusion This is the first case report of VAMP2 variant in Japan. Hypotonia at early infancy, poor visual fixation, and absence of purposeful hand movements may be indicative of the diagnosis for VAMP2 variant.

A 7-week-old girl with a normal birth history suddenly developed respiratory distress while feeding. Cardiopulmonary resuscitation was initiated at home after she had a cardiac arrest and was continued in the emergency room but all efforts at resuscitation proved unsuccessful and she died 2 h after presentation. Investigations performed in the emergency room revealed that she had a significantly high white blood cell count and severe anaemia. The cause of death was identified as KMT2A-rearranged infantile acute lymphoblastic leukaemia based on cytogenetic tests. She had no abnormalities at the 4-week check-up; however, she developed a skin nodule on her abdomen thereafter, and the family did not consult a doctor for fear of contracting COVID-19. Early detection and diagnosis could have changed the prognosis of the patient. The present case highlights the negative impact of the reduction of outpatient consultations during the COVID-19 pandemic.

Mutations in the mitochondrial tRNAMet gene have been reported in only five patients to date, all of whom presented with muscle weakness and exercise intolerance as signs of myopathy. We herein report the case of a 12-year-old girl with focal epilepsy since the age of eight years. At age 11, the patient developed sudden visual disturbances and headaches accompanied by recurrent, stroke-like episodes with lactic acidosis (pH 7.279, lactic acid 11.6 mmol/L). The patient frequently developed a delirious state, exhibited regression of intellectual ability. Brain magnetic resonance imaging revealed high-intensity signals on T2-weighted images of the left occipital lobe. Mitochondrial gene analysis revealed a heteroplasmic m.4450G > A mutation in the mitochondrial tRNAMet. The heteroplasmic rate of the m.4450G > A mutation in blood, skin, urinary sediment, hair, saliva, and nail samples were 20, 38, 59, 41, 27, and 35%, respectively. The patient’s fibroblast showed an approximately 53% reduction in the oxygen consumption rate, compared to a control, and decreased complex I and IV activities. Stroke-like episodes, lactic acidosis, encephalopathy with brain magnetic resonance imaging findings, and declined mitochondrial function were consistent with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. To our knowledge, the findings associated with this first patient with MELAS syndrome harboring the m.4450G > A mutation in mitochondrial tRNAMet expand the phenotypic spectrum of tRNAMet gene.

BackgroundCongenital central hypoventilation syndrome (CCHS) is a rare disease characterized by sleep apnea. Anoxia often occurs soon after birth, and it is important to prevent anoxia-mediated central nervous system complications; however, data on the relationship between respiratory management and the prognosis for intellectual development of patients with CCHS is not well yet investigate.MethodsWe performed a retrospective chart review cohort study of patients with CCHS in Japan. We investigated the risk and prognostic factors for developmental outcomes and examined the disease in terms of its symptoms, diagnosis, complications, and treatment.ResultsOf the 123 patients with CCHS included in the survey, 88 patients were 6 years old and older. They were divided into two group based on their intelligence quotient. Those treated using positive-pressure ventilation via tracheostomy in the first three months of life had a better developmental prognosis than those managed via tracheostomy after three months of age and those treated by ventilation using mask (OR = 3.80; 95% CI: 1.00–14.37, OR = 4.65; 95% CI: 1.11–19.37). There was no significant difference in physical development (P = 0.64).ConclusionsThe best respiratory treatment for patients with CCHS is ventilation via tracheostomy, initiated ideally before the age of three months.

Ring chromosome 20 syndrome is an epileptic and neurodevelopmental encephalopathy that occurs in children, characterised by a triad of refractory frontal lobe seizures, recurrent non-convulsive status epilepticus and frontal lobe-dominant paroxysmal discharges. However, details of other clinical features associated with ring chromosome 20 syndrome remain unknown. Here, we report two patients with ring chromosome 20 syndrome who had praxis-induced reflex seizures. Case 1 was an 11-year-old girl who presented with seizures triggered by specific activities such as mental and written calculations, writing, decision-making, recall, sudden changes in routine or ambient temperature and bathing. During calculations, left frontal lobe-dominant, 3-Hz slow-wave bursts were observed on EEG. Lacosamide effectively suppressed her tonic seizures. Case 2 was a six-year-old boy who presented with seizures triggered by specific activities such as calculations, recall and bathing. During calculations, frontal lobe-dominant, 3-Hz spike and slow-wave bursts were observed on EEG. Although his epilepsy was refractory, gabapentin reduced the frequency of focal seizures. In both cases, the hyperexcitability in the frontal lobe may have spread to the motor cortex and precipitated praxis-induced seizures. Therefore, in addition to the known characteristic triad, praxis-induced reflex seizures may also be a feature of ring chromosome 20 syndrome.

The application of fault current limiter is an effective method to limit fault current. The saturated-core fault current limiter (SFCL) is one of the promising devices. However, its commercialization is limited by the high demands for the ferromagnetic material and the dc source generating magneto motive force. This paper proposes a novel three-phase compact SFCL (CSFCL) in which a common core and a permanent magnet are used to solve the mentioned challenges. The common core can significantly reduce the amount of required ferromagnetic material. The basic operating principle of CSFCL is introduced based on the analysis of the CSFCL’s magnetic circuit and equivalent electrical circuit. Finite-element analysis (FEA) simulations were performed by ANSOFT to validate the effectiveness of the CSFCL’s fault clipping performance.

Herein we report the case of a 6-year-old girl with autism spectrum disorder (ASD) and weakness in the distal portion of the right upper limb. Although difficult to perform, nerve conduction studies indicated demyelinating neuropathy. Magnetic resonance imaging (MRI) showed swelling a nd high-intensity signals in the right brachial plexus and cervical spinal roots. The symptoms recovered after a single course of i.v. immunoglobulin. Electrophysiological indices and MRI findings also improved after treatment. This case demonstrates the utility of neuroimaging in addition to electrophysiological assessments for the diagnosis of demyelinating neuropathy, particularly in young patients with ASD.

When a magnetically shielded room (MSR) to shield leakage fluxes of electric machines is designed by using magnetic field analysis, it is difficult to represent the leakage magnetic field of an electric machine accurately. In this paper, a method of modeling it using a simple solid model with multiple magnetization vectors is proposed. In this method, the magnetization vectors are determined by an inverse technique to represent a real leakage flux distribution. First, the proposed method is explained and verified using a simple 2-D linear magnetostatic one-phase transformer model. Then, the method is applied to a 3-D model and the effect of selected measured surface on representation of leakage flux is investigated. Finally, an experimental verification is carried out. It is shown that the proposed method can represent the leakage magnetic field with small number of magnetizations. The leakage flux only in the region behind the measured surface can be represented, and it is verified by experiment. Moreover, the shielding performance of MSR can be evaluated by using the proposed solid model with suitable permeability.

Eddy-current loss of NdFeB permanent magnets (PMs) is widely analyzed in PM motors. This paper proposes a coupled 2-D transient finite-element analysis (FEA) of the saturated core fault current limiter (SCFCL) and 3-D analytical model of PMs to evaluate eddy-current loss in SCFCL. The comparison between the result from coupled method and that from 3-D FEA verifies the applicability of the coupled method in analyzing eddy current loss of PMs in SCFCL. The 3-D analytical model of PM is applied to analyze the effect of segmentation on the reduction of PM's eddy-current loss. And the results show that segmentation along width has a better performance, which is also verified by 3-D FEA.

Introduction Levetiracetam has a high tolerability and is effective against various seizure types and epilepsy syndromes. However, no study has specifically evaluated the efficacy of levetiracetam in children with refractory epilepsy based on magnetic resonance imaging (MRI) findings and the presence of intellectual disability (ID). Methods We retrospectively evaluated levetiracetam efficacy and safety in 49 pediatric patients who met the following inclusion criteria: (1) diagnosis of refractory epilepsy with first-line antiepileptic (AED) treatment ⩾2 years, (2) younger than 20 years old, and (3) received oral levetiracetam treatment for ⩾6 months. We assessed the relationships of these outcomes with MRI findings and ID status. Results Eighteen (37%) patients achieved a ⩾50% reduction in seizure frequency, and the majority (78%) had no remarkable side effects. Twenty-two (45%) patients had previously been treated with more than seven antiepileptic drugs prior to levetiracetam. Among 18 patients who achieved a ⩾50% reduction in seizure frequency, 13 and 5 had negative and positive MRI findings, and 9 and 9 had and did not have ID, respectively. Conclusions Our findings suggest that even for intractable pediatric cases with symptomatic etiology (i.e., MRI lesion and ID), levetiracetam has favorable efficacy for refractory epilepsy with tolerable adverse effects.

Introduction Neuronal ceroid lipofuscinoses (NCLs; CLN) are mainly autosomal recessive neurodegenerative disorders characterized by the accumulation of autofluorescent lipopigments in neuronal and other cells. Symptoms include visual disabilities, motor decline, and epilepsy. Causative genes are CLN1, CLN2, CLN3, CLN5, CLN6, CLN7, CLN8, CLN10, CLN11, CLN12, CLN13, and CLN14. We present the fourth Japanese case with a CLN6 mutation. Case presentation At 3 years of age, our patient became clumsy and fell down easily. He developed focal seizures with impaired consciousness and was started on carbamazepine. He showed ataxic walking and dysarthria with increased deep tendon reflexes. Interictal electroencephalogram revealed slow waves in the left temporal and occipital areas. Brain magnetic resonance imaging showed cerebellar atrophy and ventriculomegaly. In optical coherence tomography (OCT), the inner layer of the retina was thick and highly reflective. Exome sequencing revealed a known homozygous mutation, C.794_976del, p. (Ser265del) in CLN6. Discussion A total of 130 cases of NCL with CLN6 mutations have been reported globally, of which only four were from Japan including the current patient. The deletion of serine at position 265 has been reported in six cases. Ser265 is located in a region of short repeated sequences that is susceptible to mutation. Clinical trials of gene therapy using adeno-associated virus serotype 9 have started for NCL6, making early diagnosis crucial. OCT examination might be helpful in achieving a diagnosis.

In this paper, we present a dual lock system using radio-frequency identification (RFID) and fingerprint recognition. It is a secure and reliable locking system which can be implemented in home, offices, school and in any kind of organization. Even if the users lost the RFID card, they can unlatch the lock with their fingerprints. Only the registered users are able to access using their fingerprints and the RFID tags. If any unauthorized persons try to have an access, first of all short message service (SMS) will send a text message to an owner using global system for mobile communications (GSM), and at the same time a TTL camera will take a snap of that person and store it in a SD card for reference of the owner. The core of the system is the Arduino Mega 2560 which is interfaced with a fingerprint scanner module, a RFID card reader module, the TTL camera, an SD card module, a GSM shield, a liquid crystal display (LCD) and an electric door strike.

Design education is important at technical universities and colleges. In general, real product design requires collaborative work. In this chapter, the authors discuss collaborative design education. An A360 cloud platform on Autodesk's 3D computer-aided design “AutoCAD” is adopted to illustrate a collaborative design activity implemented in the Engineering Graphics class offered at the College of Science and Technology, Royal University of Bhutan. By using A360 cloud, students can share a 3D model with group members. Based on feedback received, students can modify the initial model, share it, print, and discuss the modified object with members. This collaborative work allows students to create enhanced 3D design objects while engaged in discussions and interactions. The authors also discuss some difficulties encountered during the collaborative process and offer recommendations and future research ideas.

With the rapid development of the power system, the fault current becomes a big problem. The current limiting measures become more and more important. The saturated-core fault current limiter(SFCL) is one of the promising methods [1]. The most serious fault in electric power system is three-phase short-circuit fault, and the fault current is more likely to exceed the circuit breaker's capacity. The use of three single-phase current limiters at the same time will need a lot of ferromagnetic material and space. A compact three-phase SFCL was proposed in [2], but it cannot limit three-phase fault current [2]. So we propose a novel structure of Economical Three-phase Saturated-core Fault Current limiter(ETSFCL). The novel structure has good clipping performance at three-phase fault conditions. Compared to three single-phase SFCLs proposed in [3], it saves a lot of ferromagnetic material and reduces the AC flux through the permanent magnet. The stability of the permanent magnet is greatly improved and the risk of demagnetization is reduced. In this paper, the operating principle of the proposed structure is explained by analyzing its equivalent magnetic circuit and electrical circuit. FEA models were established in ANSOFT to verify the its effectiveness.

The start current can reach up to as high as 4–7 times of the motor's rated current when high voltage motors start directly. For super large capacity motors (voltage above 6 kV, power above 10000 kW), the direct start current could reach 5000 Ampere or more [1]. Such large start current will not only have a huge impact on the motor itself but also cause a sudden decline of the grid voltage [2], [3]. In order to resolve this issue, a novel coupled auto-transformer and magnetic control (CATMC) soft starter method was proposed in this paper. The new structure of CATMC soft starter combines the two functions of the auto-transformer and the magnetic control reactor (MCR) through ingenious electric and magnetic circuit design. Hence the start current is reduced in two ways and the structure becomes more compact and flexible.

The rapid progress in and the expanding complexity of information and technology systems have made data analysis increasingly relevant. Data having a variety of elements are complex, and making very difficult to evaluate a state of a model from observed data generated probabilistically by the model. To evaluate these hidden states, we propose Spherical-Self Organizing Map (S-SOM) with a Hidden Markov Model (HMM) that infers such hidden states.

Introduction The relevant literature includes several case reports on cerebral infarction in children with HHV-6 infection; however, there is no report of brain stem infarction. Case An 11-month-old girl was hospitalized because of fever. She was unable to stand up and meet her mother’s gaze. Magnetic resonance imaging (MRI) indicated a right pons and mid-brain lesion; a diagnosis of brainstem infarction was made. After her fever subsided, a rash developed on her trunk and limbs; blood examination results indicated a primary HHV-6 infection. She was treated with aspirin, edaravone, and mannitol to prevent further complications. At the age of 18 months, the auditory brainstem response (ABR) was unremarkable and she is developing well. Discussion and conclusion A limited number of studies have reported HHV-6 infection-associated infarction, and no cases of brainstem infarction have been reported. One possible cause of cerebral infarction is antiphospholipid antibody syndrome (APS) triggered by the infection. HHV-6 may also directly infect vascular endothelial cells and cause angiopathy. However, the real mechanism of infarction remains unclear. Our patient had a favorable prognosis despite brainstem infarction.

Due to the increasing demands for electric energy and installation of distributed generation, the rising fault current poses a great threat to those existing power system components.

Background and Aim The Montreal Cognitive Assessment (MoCA) is the most suitable measure for screening cognitive impairment in Parkinson's disease (PD). However, the utility of the MoCA has not been documented sufficiently, especially in Asian populations. The present multicenter study included a large number of Japanese patients, and compared Mini-Mental State Examination (MMSE) and MoCA scores in PD patients. Methods We carried out a cross-sectional study. Idiopathic PD patients (n = 304; age 70.6 ± 8.3 years (mean ± SD); disease duration 6.6 ± 5.1 years; Hoehn and Yahr stage 2.7 ± 0.7) were registered at 13 participating hospitals, and their clinical/neurological/cognitive features were examined using Japanese versions of the MMSE and MoCA. Results The MMSE and MoCA scores were 26.3 ± 3.6 (range 12–30) and 20.9 ± 5.0 (range 5–30), respectively, and showed a strong correlation (R2 = 0.74, P

Idiopathic restless legs syndrome (RLS) has a genetic basis wherein BTBD9 is associated with a higher risk of RLS. Hemodialysis patients also exhibit higher rates of RLS compared with the healthy population. However, little is known about the relationship of BTBD9 and end-stage renal disease to RLS pathophysiology. Here we evaluated sleep and leg muscle activity of Btbd9 mutant (MT) mice after administration of serum from patients with either idiopathic or RLS due to end-stage renal disease (renal RLS) and investigated the efficacy of treatment with the dopamine agonist rotigotine. At baseline, the amount of rapid eye movement (REM) sleep was decreased and leg muscle activity during non-REM (NREM) sleep was increased in MT mice compared to wild-type (WT) mice. Wake-promoting effects of rotigotine were attenuated by injection of serum from RLS patients in both WT and MT mice. Leg muscle activity during NREM sleep was increased only in MT mice injected with serum from RLS patients of ideiopatic and renal RLS. Subsequent treatment with rotigotine ameliorated this altered leg muscle activity. Together these results support previous reports showing a relationship between the Btbd9/dopamine system and RLS, and elucidate in part the pathophysiology of RLS.

In this paper, the play model and the free energy model are applied to represent the hysteretic properties of non-oriented steel plates and compared. The ac symmetric B-H loops and PWM minor loops are represented by both methods and the results are compared with the measured ones. Small minor loops representation is found not complete with both methods. However, in play model, it can be easily improved by increasing the number of hysterons. In the spring model, the way to improve the minor loop representation accuracy is under discussion, whereas its identification is simpler than play model as less parameters are required.

The Multi-magnetic-valve structure is adopted in Magnetic Controlled Reactors (MCR) to reduce their current harmonics. However, the reactance can't be precisely calculated for its complicated structure. In this paper, an improved method to calculate its inductance is presented and the simulation analysis based on finite element method (FEM) verified its validity. The analysis results demonstrate that the length and the number of magnetic valves have a great impact on the inductance of MCR.

Due to the leakage flux effect, the nonuniform magnetic flux distribution would weaken the biasing ability of PMs in a permanent-magnet-biased fault current limiter. To improve the biasing ability of PMs and reduce the cost, a novel small-section permanent-magnet-biased fault current limiter is proposed by this paper. Firstly, the principle and the optimal small-section structure are introduced. To validate the impact of small section on the biasing ability of PMs, various FEA simulation and optimization study are performed in Ansoft. The simulation and optimization results verify that the small section can improve the biasing ability of PMs and reduce the cost.

Eddy current loss of NdFeB permanent magnets (PMs) are widely analyzed in permanent magnet motors. And it's necessary to analyze this issue in saturated core fault current limiter (SCFCL). This digest discusses the coupled 2-D finite element analysis (FEA) of the SCFCL and 3-D analytical model of PMs to evaluate the eddy current loss of PMs in a SCFCL. The comparison between result from coupled method and that from 3-D FEA verifies the applicability of the coupled method in analyzing eddy current loss of PMs in SCFCL.

In allusion to the difficulties in the optimal gear capacity design of the superconducting controllable reactor (SCR), a novel design optimization method of SCR is proposed in this paper combining field-circuit coupling finite element method (FEM) and Immune Algorithm (IA). Field-circuit coupling finite element method is realized in ANSYS and immune algorithm is embedded in ANSYS by ANSYS Parameter Design Language (APDL). The proposed method and the conventional method will make a detailed comparison. Finally, a 380V/30 KVar SCR prototype is fabricated, and detailed experiments are done.

To reduce the size and requirement of DC biasing capacity of saturated-core fault current limiter, a novel hybrid compact saturated-core fault current limiter (HCFCL) is proposed by this paper. The three-limb structure with Permanent magnets can reduce the size and DC biasing capacity. Firstly, the principle of HCFCL is introduced. To validate the performance, various FEA simulations with the 3D nonlinear field-circuit coupling transient analysis are carried out in Ansoft. And a 220V/10A prototype of HCFCL is designed and tested. The simulation and experimental results verify the effectiveness and excellent performance of proposed structure.

In this paper, a novel hybrid saturated-core fault current limiter (HSFCL) is proposed considering the reliability of permanent magnets (PMs). For the HSFCL, a hybrid PMs/DC coil is designed to saturate the core and a fault limiting reactor is used to guarantee the fault clipping performance. The method of electromagnetic coupling is used to analyze the HSFCL based on ANSYS Maxwell. Simulation results have demonstrated that the HSFCL is efficient in fault clipping and stability enhancement of PMs.

This digest proposed a model of superconducting controllable reactor and calculates its reactance based on finite element method. In order to ensure the safe operation of superconducting reactor, the digest devises a control system for superconducting windings based on S7-200 PLC after analyzing the DC component and overvoltage of superconducting winding and puts forward an optimal control strategy. A 380V/30kVar SCR was fabricated for experiments and the experimental results show the correctness of this method and the feasibility of the control device.

Excess loss evaluation in SiFe electrical steel sheets is important for accurate iron loss prediction of electrical machines, especially under high frequencies. In this paper, a numerical excess loss estimation method of SiFe sheets considering the domain wall bowing caused by pinning sites is proposed. An equivalent magnetic field representing the pinning effect is implemented into a general nonlinear eddy current finite element analysis. The hysteresis and eddy current losses are calculated using the flux and eddy current distributions obtained from the proposed analysis directly. The proposed method is applied to two different grades SiFe sheets first. The calculated iron losses under various frequencies in one sheet are compared with catalogue data and less than 2% deviation can be achieved.

Delirious behavior associated with influenza usually has an onset within a few days after fever and lasts 3 days after fever) and long-standing (>48 hours) delirious behavior. Each exhibited mild to moderate drowsiness between the episodes of delirious behavior. Electroencephalography was normal except for 1 patient with high voltage and slow activity bilaterally in the occipital regions. Brain MRI was normal. The outcome was excellent with no neurologic sequel in 4 of the 5 patients. In all 5 patients, autoantibodies against N-methyl-D-aspartate type glutamate receptor were elevated or positive in cerebrospinal fluid or serum; the autoantibody levels normalized in the 3 patients who had follow-up studies. This study indicates that 2009 H1N1 influenza has a tendency to cause late-onset and long-standing delirious behavior, at least in Japanese children. Mild autoimmune-mediated encephalitis should be considered as an underlying cause.

Objective: The aim of this work was to investigate the prevalence of camptocormia and the clinical characteristics of patients with camptocormia in a large population of PD patients. Background: Although camptocormia has been recognized as a prominent phenomenon in PD, the previous epidemiological reports were limited, especially in terms of sample size. Methods: We evaluated 531 PD patients (disease duration: 7.0 ± 5.5 years, mean ± standard deviation). We examined their clinical features and the prevalence of camptocormia. Results: Camptocormia was detected in 22 patients (4.1%) and found in patients who were older and had more severe motor symptoms and a higher levodopa (L-dopa) dose (P < 0.05), compared to the patients without camptocormia. Patients with camptocormia showed significantly higher frequencies of autonomic symptoms, such as constipation and urinary incontinence (P < 0.05). Conclusions: Camptocormia is uncommon in PD and is associated with disease severity, higher L-dopa dose and higher frequencies of autonomic symptoms. © 2011 Movement Disorder Society

眼球運動障害の所見から虚血範囲を経時的に評価し得た脳幹梗塞について報告する．症例は67歳男性，2007年5月某日に左側方注視麻痺を自覚し来院した．左側方注視麻痺および右上下肢の異常感覚，右第5指徴候を認めたものの，頭部MRIでは異常所見はなかった．症状はその後改善したが，臨床的に脳幹梗塞または痙攣発作が疑われたため入院した．第1病日夜間に両側方注視麻痺と，四肢麻痺が出現した．この時の頭部MRI拡散強調画像(diffusion-weighted image：DWI)で両側延髄上部腹側から正中背側に達する梗塞を認めた．第2病日に四肢麻痺は改善した．その後，両側MLF症候群，さらに左MLF症候群を経て第11病日には眼球運動障害も消失した．第23病日の頭部MRI T2強調画像では病巣は橋下部被蓋部に達していた．急性期脳幹梗塞はDWIでも陽性所見の出現が遅れることが多いが，本例の虚血病変が橋被蓋に存在したことは入院時および入院後の眼球運動障害により推測し得た．脳幹には眼球運動を司る神経線維や神経核が密に存在し，脳幹梗塞を疑う患者において眼球運動の詳細な観察は，虚血巣の進行をMRIよりも経時的に評価しうると考えられた．

To improve the comfort of hospital patients, we have developed a new open-type magnetically shielded room (MSR) for magnetic resonance imaging (MRI), constructed by stacking square cylinders composed of magnetic material on the inside and a conductive material on the outside. The magnetic cylinder shields against leakage of the magnetic field from the MRI, while the conductive outer cylinder prevents intrusion of the electromagnetic field from outside by waveguide attenuation. First, the optimal design of the magnetic cylinder was determined, using 3-D magnetic field analysis with a simple cubic MSR. Second, the validity of the analysis and the practical realization of the designed MSR were investigated using a small experimental model. Finally, from a design perspective and the standpoint of saving space, the optimal combination of electromagnetically shielded glass and conductive cylinders made of aluminum was studied by measuring the electromagnetic shielding effect. As the optimal design of an open-type MSR for an MRI, we have adopted a combination of single-layered electromagnetically shielded glass together with stacked cylinders. These cylinders are made of a square cylinder, 144 x 144 mm and 300 mm long, made of silicon steel (thickness t =1 mm) and divided into three parts along the axial direction, placed inside a second square cylinder made of aluminum, 150 x 150 mm and 300 mm long (t = 3 mm). This MSR construction technique creates an open, spacious feeling inside the facility and can be easily fabricated at low cost.

Today, we are able to easily obtain data such as stock prices and market information through media such as the Internet. However, it is not so easy to come by useful information from this data. The reason for this is that there is a mix of many different kinds of information. Such a situation leads to difficulty in grasping trends in the data through just one rule, and so one must find a rule for each relevant factor. Therefore, the need for finding relevant factors is inevitable. For this reason, in our research we developed an algorithm that enables one to extract factors that have causal relationships with one another, and indicated its usefulness through experiments.

The hypothesis was tested among 83 patients with multiple lacunar infarctions that cerebral hypoperfusion will correlate with cognitive impairments. Patients were subdivided according to Cognitive Capacity Screening Examination (CCSE) scores into a cognitively impaired group (Group D, n = 40; mean age, 68.2 years) with CCSE scores between 6 and 25 (mean, 19.9) and a cognitively intact group (Group I, n = 43; mean age, 66.0) with normal scores (mean, 29.4). Gray and white matter tissue densities were measured by plain computed tomography (CT), and their compartmental perfusions were estimated during stable xenon inhalation. Eighty infarcts in basal ganglia and white matter were detected in Group D and 62 in Group I. Cognitive impairments correlated with (a) multiplicity and bilaterality of lacunes; (b) hypertension, diabetes mellitus, and multiplicity of risk factors for stroke; (c) hypoperfusion of white and gray matter, but particularly of frontal white matter; (d) leuko-araiosis; (e) aging; and (f) lower education. The conclusion was that hypertension and diabetes mellitus are potent risk factors for cerebral small vessel disease or arteriolosclerosis ultimately resulting in lacunar infarcts, leuko-araiosis, white matter hypoperfusion, and impaired cognitive test performance.

A highly practical method for evaluating magnetic disturbance due to adjacent buildings for the design of a magnetic testing site with nearly zero magnetic field was proposed. A modeling technique for complicatedly distributed magnetic materials in buildings was verified by the measurement using an actually constructed building. Moreover, a convenient formula was conceived, in which a building is replaced simply into a brick magnetic body and the flux distribution due to its magnetization is calculated analytically. It was shown that the proposed formula is useful for investigating the effect of the positions of buildings for additional construction on the magnetic disturbance.

The purpose of this study is to determine the nonlinear vibration characteristics of a spring in order to analyze the vibration response of a vehicle. In the present paper, we constructed the hysteresis restoring force models dependant on amplitude and frequency from experimental results of a dynamic hysteresis test of the tapered leaf springs, and the spring constants and damping coefficients could be calculated by using a power-function-type restoring force model. Finally, we proved the accuracy of this method by directly comparing the analytical results with the experimental results on the vibration characteristics (spring constant, damping coefficient). Using the above method, it will become possible to calculate more precisely the nonlinear vibration response of vehicles.