門田 行史

Seizures in patients with developmental and epileptic encephalopathies (DEEs) are often highly resistant to various antiseizure medications. Perampanel (PER) is a novel antiseizure medication that non-competitively inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor and is expected to reduce seizure frequency not only for focal seizures and generalized tonic-clonic seizures (GTCS) but also for other seizure types. This study aimed to clarify the long-term therapeutic efficacy and tolerability of PER in patients with DEEs. We analyzed data regarding patients' background characteristics, medication retention, trends in seizure frequency, and adverse events obtained from 24 patients with DEEs who had been on PER treatment for 60 months. The retention rates were 62.5% and 46.9% at 12 and 60 months, respectively. At 60 months after PER initiation, the rate of patients with > 50% seizure reduction was 33.3%, 33.3%, 38.5%, 54.5%, 54.5%, and 36.4% among patients with atypical absence seizures, tonic seizures, focal seizures, GTCS, myoclonic seizures, and atonic seizures, respectively. The frequency of adverse events was 70.8%. PER showed long-term efficacy in various seizure types. PER is a promising treatment option for patients with DEEs.

OBJECTIVE: This study undertook neuropharmacological research on the clinical course of controlled medication discontinuation to guide practitioners who are considering stopping medications for youths with attention-deficit hyperactivity disorder (ADHD). METHODS: This study analyzed the data for 14 ADHD children (12 male and 2 female) in two datasets: The children prescribed methylphenidate (MPH) were at an initial mean age of 7.5 years (SD = 1.70, range: 6-11) with a mean ADHD-Rating Score (ADHD-RS) of 26.6 (SD = 8.64, range 15-40). The children who discontinued MPH based on clinical judgment were at a mean age of 12.21 years (SD = 2.12, range: 8-15) with a mean ADHD-RS of 15.9 (SD = 6.86, range 5-27). The go/no-go task was used to assess response inhibition, while functional near-infrared spectroscopy (fNIRS) was used to measure cerebral hemodynamics. Oxygenated hemoglobin (Oxy-Hb) values from fNIRS data were analyzed for each subject, focusing on past and current measurements. Baseline was set at 10 s pre-task, with interval means from 4 to 24 s analyzed. One-sample t-tests were used to evaluate brain activity magnitude. RESULTS: The results of the study demonstrate that the children who had discontinued the medication exhibited activation in specific brain regions including the frontopolar cortex and the right ventrolateral prefrontal cortex. Activation (t = 2.363, p = 0.034, Cohen's d = 0.632) was found especially in the right dorsolateral prefrontal cortex during the performance of the go/no-go task. These activated areas were consistent with those observed in a previous study comparing brain activity during a go/no-go task between children with ADHD and healthy children. CONCLUSION: The present study showed differences in cerebral hemodynamics before and after discontinuation of MPH in ADHD children whose ADHD symptoms did not recur after MPH was discontinued. In the near future, further investigations that include control groups will be conducted to demonstrate the effects of MPH prior to discontinuation based on the changes in cerebral blood flow in the right prefrontal cortex, which is involved in behavioral inhibition, as observed in this study. This and future research will facilitate the development of criteria for discontinuing treatment.

BACKGROUND: Behavioral problems of foster children are an important issue for the maintenance of the foster care system, but they have not been adequately studied in Japan. We used the Eyberg Child Behavior Inventory (ECBI) to investigate behavioral problems among foster children and to examine associated factors. METHODS: Twenty-nine foster children and their foster parents and 479 non-foster children and parents were recruited for the foster and control groups, respectively. Both groups underwent statistical comparative analyses using data from their ECBI assessments. The ECBI has two scales: the Intensity Scale quantifies the severity of child behavioral problems, and the Problem Scale captures the caregiver's perceived difficulties handling each behavior. We conducted a retrospective investigation of the background of the foster parent-child pairs to explore potential causal relationships with behavioral problems. RESULTS: The mean intensity score for the foster group was significantly higher than that for the control group (p = 0.001). The mean problem scores for the foster group and the control group were not significantly different (p = 0.79). In the foster group, the retrospective investigation revealed two children with neurological or neurodevelopmental disorders, 17 with histories of abuse, and 10 with other issues. CONCLUSION: Intensity scores showed severe behavioral problems among foster children, perhaps caused by neurological disorders, abuse, parental mental health, or economic hardship. Problem scores showed no significant differences between groups. It can therefore be posited that foster parents might exhibit a more lenient parenting style when dealing with children who have a history of abuse by their biological parents.

<jats:title>Abstract</jats:title><jats:p>Difficulties with visual perspective-taking among individuals with autism spectrum disorders remain poorly understood. Many studies have presumed that first-person visual input can be mentally transformed to a third-person perspective during visual perspective-taking tasks; however, existing research has not fully revealed the computational strategy used by those with autism spectrum disorders for taking another person’s perspective. In this study, we designed a novel approach to test a strategy using the opposite-directional effect among children with autism spectrum disorders. This effect refers to how a third-person perspective as a visual input alters a cognitive process. We directly manipulated participants’ visual perspective by placing a camera at different positions; participants could watch themselves from a third-person perspective during a reaching task with no endpoint feedback. During a baseline task, endpoint bias (with endpoint feedback but no visual transformation) did not differ significantly between groups. However, the endpoint was affected by extrinsic coordinate information in the control group relative to the autism spectrum disorders group when the visual perspective was transformed. These results indicate an increased reliance on proprioception during the reaching task with perspective manipulation in the autism spectrum disorders group.</jats:p>

<jats:p><jats:bold>Objective:</jats:bold> In the current study, we explored the neural substrate for acute effects of guanfacine extended release (GXR) on inhibitory control in school-aged children with attention deficit hyperactivity disorder (ADHD), using functional near-infrared spectroscopy (fNIRS).</jats:p><jats:p><jats:bold>Methods:</jats:bold> Following a GXR washout period, 12 AD HD children (6–10 years old) performed a go/no-go task before and 3 h after GXR or placebo administration, in a randomized, double-blind, placebo-controlled, crossover design study. In the primary analysis, fNIRS was used to monitor the right prefrontal cortical hemodynamics of the participants, where our former studies showed consistent dysfunction and osmotic release oral system-methylphenidate (OROS-MPH) and atomoxetine hydrochloride (ATX) elicited recovery. We examined the inter-medication contrast, comparing the effect of GXR against the placebo. In the exploratory analysis, we explored neural responses in regions other than the right prefrontal cortex (PFC).</jats:p><jats:p><jats:bold>Results:</jats:bold> In the primary analysis, we observed no significant main effects or interactions of medication type and age in month (two-way mixed ANCOVA, <jats:italic>Fs</jats:italic> &lt; 0.20, all <jats:italic>ps</jats:italic> &gt; .05). However, in the <jats:italic>post-hoc</jats:italic> analysis, we observed significant change in the oxy-Hb signal in the right angular gyrus (AG) for inter-medication (one sample <jats:italic>t</jats:italic>-test, <jats:italic>p</jats:italic> &lt; 0.05, uncorrected, Cohen's <jats:italic>d</jats:italic> = 0.71).</jats:p><jats:p><jats:bold>Conclusions:</jats:bold> These results are different from the neuropharmacological effects of OROS-MPH and ATX, which, in an upregulated manner, reduced right PFC function in ADHD children during inhibitory tasks. This analysis, while limited by its secondary nature, suggested that the improved cognitive performance was associated with activation in the right AG, which might serve as a biological marker to monitor the effect of GXR in the ADHD children.</jats:p>

Intravenous corticosteroids have been regarded as the first-line therapy of anti-myelin-oligodendrocyte glycoprotein antibody (MOG-Ab)-positive acute disseminated encephalomyelitis (ADEM). While steroids are the first-choice therapy, MOG-Ab-positive ADEM has a high relapse rate. In some cases, MOG-Ab-positive ADEM relapses even in a low-MOG-Abs state. There is no evidence-based rule supporting steroid tapering. We herein report a case of MOG-Ab-positive ADEM in which recurrence was preventing by tapering steroids under MOG-Ab seronegativity confirmation. In some cases, the MOG-Ab titer may be an important index for tapering steroids to prevent relapse.