Steroid-Responsive Involuntary Movements as a Remote Symptom of Febrile Infection-Related Epilepsy Syndrome.

Febrile infection-related epilepsy syndrome (FIRES) is a rare epileptic encephalopathy that occurs in children or adolescents. To date, evidence for the management of the post-acute phase of FIRES is focused on drug-resistant epilepsy that continues from the acute phase. Information on involuntary movements, which are newly developed in the chronic phase, is limited.

Therapeutic Strategies in Children with Epilepsy: A Quality-of-Life-Related Perspective.

Children with epilepsy are affected by several factors, including clinical and social variables. Among these variables, cognitive decline and behavioral disturbances, perceptions of stigma, and fatigue can lead to reductions in quality of life (QOL). Epileptic activities, including seizure severity, frequent seizures, and status epilepticus (SE), have been identified as important predictors of QOL.

Effect of Lacosamide on Interictal Epileptiform Discharges in Pediatric Patients With Newly Diagnosed Focal Epilepsy.

Background: The purpose of this study was to determine the efficacy of lacosamide (LCM) on interictal epileptiform discharges (IEDs) and evaluate the relationships between IEDs and seizure outcome in pediatric patients with focal epilepsy.

Methods: Patient inclusion criteria included (1) newly diagnosed focal epilepsy with unknown etiology; and (2) electroencephalogram recorded twice (before and after starting LCM) under the same conditions. The difference between the highest number of IEDs over five successive minutes (IEDs/5 min) and the location of IEDs was determined.

Factors associated with the development of epilepsy in very low birth weight infants.

Background: The survival rate of very low birth weight (VLBW) infants has recently improved. However, the occurrence of and factors associated with epilepsy in VLBW infants remain unknown. This study aimed to clarify the incidence, characteristics, and factors associated with epilepsy development in VLBW infants.

ACTH for epileptic spasms in Leigh syndrome with SLC19A3 mutation can induce status dystonicus.

Patients with Leigh syndrome (LS) sometimes develop epileptic spasms (ES). ACTH treatment for ES may be effective without serious adverse events in some patients with LS. Status dystonicus is a life-threatening disorder characterized by an acute exacerbation of generalized dystonia and often develops as a triggered event.

Efficacy of perampanel in epilepsy patients with autism spectrum disorder.

Aim: The aim of this study was to assess the usefulness of perampanel (PER), and to identify the relationship between behavioral impairments and electroencephalogram (EEG) findings in epilepsy patients with autism spectrum disorder (ASD).

Methods: Participants were ASD patients with epilepsy recruited between June 1, 2016 and June 30, 2018. Inclusion criteria were: seizures refractory to two appropriate antiseizure medications (ASMs); presence of neuropsychological impairments; and ≥12 months of monitoring.

Clinically Mild Encephalopathy With a Reversible Splenial Lesion Type 2 Caused by Human Herpesvirus 6 Infection.

Background: Clinically mild encephalopathy with a reversible splenial lesion (MERS) is the second commonest cause of encephalopathy. Several pathogens have been detected in patients with MERS type 2, such as influenza A and B, but little is known about the proportion of cases of MERS type 2 with this pathogenesis. Human herpesvirus 6 (HHV6) is the second commonest pathogen causing acute encephalopathy.

Glucose transporter type 1 deficiency syndrome associated with autoantibodies to glutamate receptors.

Background: The clinical spectrum of glucose transporter type 1 deficiency syndrome (GLUT1DS) has broadened, with increasing recognition of a milder phenotype. Antibodies targeting the subunits of glutamate receptors (GluRs), including GluN1, GluN2B, and GluD2, have been detected in various neurological disorders. Anti-GluD2 antibodies in particular may be associated with cerebellar symptoms.

Effects of perampanel on secondary bilateral synchrony and behavioral problems in adolescents with epilepsy showing insufficient response with levetiracetam.

Purpose: The purpose of this study was to determine the efficacy of perampanel (PER) on secondary bilateral synchrony (SBS) and behavioral problems in adolescents with epilepsy who showed insufficient response to levetiracetam (LEV).

Methods: The primary criterion for patient selection was the presence of SBS. The criteria such as age between 12 and 18 years, seizures refractory to antiseizure medications including LEV, at least four seizures a month, neuropsychological impairments, and at least 12 months of follow-up also had to be fulfilled.

The effect of the guidelines for management of febrile seizures 2015 on clinical practices: Nationwide survey in Japan.

Objective: To investigate the effect of guidelines for management of febrile seizures on the clinical practice, we conducted a nationwide survey in Japan.

Methods: The Japanese guidelines for management of febrile seizures 2015 (GL2015) was released in 2015. In 2016, a questionnaire was sent to all 512 certified hospitals (3 pediatricians each) of the Japan Pediatric Society and all 47 prefecture Pediatric Associations (10 private pediatricians each) in Japan asking about management policies for febrile seizures (FSs) during 2013-2014 and 2016.

Usefulness of perampanel with concomitant levetiracetam for patients with drug-resistant epilepsy.

Purpose: The purpose was to evaluate the efficacy of treatment and the occurrence of aggression-related adverse events among children receiving perampanel (PER) with concomitant levetiracetam (LEV).

Methods: Patients were selected according to the following criteria: 1) between 12 and 18 years old; 2) seizures refractory to at least 2 first-line drugs; 3) at least 4 seizures a month before PER administration; and 4) at least 12 months of follow-up. Patients were subdivided into groups with and without LEV as concomitant treatment.

Efficacy of levetiracetam for reducing rolandic discharges in comparison with carbamazepine and valproate sodium in rolandic epilepsy.

Purpose: The main purpose of this study was to compare the efficacy of levetiracetam (LEV) with the older antiepileptic drugs (AEDs) for preventing atypical evolution in children with Rolandic epilepsy (RE). Accordingly, the present study compared the efficacy of older AEDs (carbamazepine (CBZ) and valproate sodium (VPA)) with LEV in reducing rolandic discharges (RDs) on interictal electroencephalogram (EEG) in children with RE.

Methods: Patients in this heterogenous study were subdivided into CBZ, VPA and LEV groups in accordance with the initial monotherapy.

Systematic review of the screening, diagnosis, and management of ADHD in children with epilepsy. Consensus paper of the Task Force on Comorbidities of the ILAE Pediatric Commission.

Attention-deficit/hyperactivity disorder (ADHD) is a common and challenging comorbidity affecting many children with epilepsy. A working group under the International League Against Epilepsy (ILAE) Pediatric Commission identified key questions on the identification and management of ADHD in children with epilepsy. Systematic reviews of the evidence to support approaches to these questions were collated and graded using criteria from the American Academy of Neurology Practice Parameter.

Association between seizure frequency and fatigue levels in children with epilepsy.

Aim: The purpose of this study was to examine the association between seizure-related features and fatigue levels in children with epilepsy.

Methods: All children were classified into three subgroups based on the state of their seizure control: well-controlled epilepsy (WCE; seizure-free), intermediate-controlled epilepsy (ICE; seizure frequency < 1×/month) and uncontrolled epilepsy (UCE; seizure frequency > 1×/month). Participants were asked to rate on a 7-point scale, from 1 (strongly disagree) to 7 (strongly agree), how often they felt the ways described by nine items on the Fatigue Severity Scale (FSS).

Effect of Levetiracetam Monotherapy in Nonlesional Focal Childhood Epilepsy.

This article compares the efficacy and tolerability of carbamazepine (CBZ) and levetiracetam (LEV) when used as initial monotherapy in children with nonlesional focal epilepsy. Patients with nonlesional focal epilepsy were subdivided into two groups according to the initial monotherapy: a LEV group administered LEV at an initial dose of 5 mg/kg/day and a CBZ group. Seizure response, adverse events, medication dose, reasons for discontinuing medication, adherence, and random serum levels were recorded.

Developmental changes in autonomic responses are associated with future reward/punishment expectations: A study of sympathetic skin responses in the Markov decision task.

Objective: Autonomic nervous system activity is recognized as a major component of emotional responses. Future reward/punishment expectations depend upon the process of decision making in the frontal lobe, which is considered to play an important role in executive function. The aim of this study was to investigate the relationship between autonomic responses and decision making during reinforcement tasks using sympathetic skin responses (SSR).

[The efficacy of intravenous alendronate for osteoporosis in patients with severe motor intellectual disabilities].

Objective: Bone fractures in patients with severe motor and intellectual disabilities (SMIDs) have become an important problem to be solved. These fractures may result from disuse osteoporosis. Bisphosphonate administration is generally the most established treatment for patients with osteoporosis.

Disinhibition in children with attention-deficit/hyperactivity disorder: Changes in [oxy-Hb] on near-infrared spectroscopy during "rock, paper, scissors" task.

Objective: Attention-deficit/hyperactivity disorder (AD/HD) is a common developmental disorder. Many reports have suggested that symptoms of AD/HD are related to frontal lobe dysfunctions, particularly disinhibition. However, measuring neurological findings with biomarkers during frontal functional tasks has sometimes been difficult in children with AD/HD.

Developmental changes in autonomic emotional response during an executive functional task: A pupillometric study during Wisconsin card sorting test.

Objective: The autonomic nervous system has a deep relationship with the cognitive network when performing cognitive tasks. We hypothesize that autonomic emotional responses can affect cognitive function, especially executive function. The aim of this study was to clarify the involvement of the autonomic system during an executive functional task via developmental changes assessed using pupillometry.

New guidelines for management of febrile seizures in Japan.

In 2015, the Japanese Society of Child Neurology released new guidelines for the management of febrile seizures, the first update of such guidelines since 1996. In 1988, the Conference on Febrile Convulsions in Japan published "Guidelines for the Treatment of Febrile Seizures." The Task Committee of the Conference proposed a revised version of the guidelines in 1996; that version released in 1996 was used for the next 19years in Japan for the clinical management of children with febrile seizures.

Seizure severity in children with epilepsy is associated with their parents' perception of stigma.

Aims: To develop and implement interventions to improve the quality of life (QOL) in children with epilepsy, it is important for clinicians and researchers to understand the effects of the children's parents' perception of stigma. The purpose of this study was to identify a relationship between patient clinical characteristics and perception of stigma in the parents of children with epilepsy.

Methods: Parents of children with epilepsy were recruited from our university hospital between April 1, 2005 and March 31, 2012.

[A case of acute encephalopathy presenting with transient functional disability in various new tasks and hypoperfusion of the right frontal and temporal lobes on SPECT].

We report the case of a 5-year-old boy with acute encephalopathy presenting with transient executive dysfunction such as functional disability in various new tasks and hypoperfusion of the right frontal and temporal lobes on single photon emission tomography (SPECT). He presented with a 2-day history of disturbed consciousness, and electroencephalography in an awaked state showed diffuse high-voltage slow waves. Although MRI did not show any abnormality 3 days after initial onset of illness, SPECT showed hypoperfusion of the right frontal and temporal lobes at the same time.

Effect of Sunlight Exposure on Bone Mineral Density in Children with Severe Disability.

The aim of this study was to determine the efficacy of sunlight exposure for increasing bone mineral density (BMD) in children with severe disability. The subjects were five children with severe disability, aged 6 to 8 years. BMD was measured at baseline and after 3, 6, 9, and 12 months of starting sunlight exposure.