門田 行史

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.

Abstract Background Infants with trisomy 13 have a very high mortality rate. However, aggressive interventions for their complications, can improve their prognosis and may, thereby, increase the number of long‐term survivors with trisomy 13. To date, there is no study on the psychomotor developmental progress of patients with trisomy 13. We conducted this survey to clarify the prognostic factors, living circumstances, and developmental status of infants the trisomy 13. Methods Patients with trisomy 13 who were admitted to the Department of Pediatrics, Jichi Medical University Hospital were enrolled. Their clinical data were investigated retrospectively using clinical records. Results Nine patients with trisomy 13 were enrolled and divided into the early death (died at <1 year) and long‐term survival (survived for >1 year) groups. All the early death group patients had severe congenital heart disease. Heart failure at under 1 year of age was associated with early death. All the long‐term survival group patients underwent operations (e.g. tracheostomy or gastrostomy) and all used home nursing and/or a social care service. Three patients used home mechanical ventilation. None of the patients was able to stand alone or speak intelligible words. Two patients without severe brain anomalies were able to roll over, sit up, and smile by 3 years of age. Conclusions Long‐term survivors with trisomy 13 require extensive nursing and medical care. It is important to provide medical and welfare services to reduce the burden on families. In patients without severe brain anomalies, psychomotor development may be expected. However, no clear developmental prognostic factors were found.

症例は神経発達障害を呈した11歳の男児で、8歳時に撮像したMRI画像で、大脳白質の髄鞘形成不全が認められたが、大脳基底核や小脳の萎縮はみられなかった。また、10歳時には軽度の知的障害(IQ=52)と診断され、11歳時に撮像したMRI画像では小脳の髄鞘形成不全と萎縮が認められたが、大脳基底核の大きさは正常であった。これらの所見から、全エクソームシーケンシングによる遺伝子解析を行ったところ、TUBB4A遺伝子にde novoヘテロ接合性バリアントc.1088T>C, p.(Met363Thr)が検出された。本例から、TUBB4A遺伝子に病的バリアントを有する患者には小脳の脆弱性が認められるという仮説が支持された。

<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>

急性呼吸不全が原因で入院を繰り返す重症心身障害児に対し、在宅非侵襲的陽圧換気療法(noninvasive positive pressure ventilation;NPPV)を呼吸状態の安定時に使用する効果について検証した。重症心身障害児3例を対象に、在宅NPPV導入前後の入院回数を後方視的に比較した結果、在宅NPPV導入前の5年間で、全症例において呼吸障害を原因とする入院が5～17回であったが、導入後は入院回数が0回となり、在宅NPPVの高い有効性を示した。また、全症例で在宅NPPV導入前に気管切開術の実施を家族と協議したが、気管切開術の高い侵襲性のため実施に至らなかったが、在宅NPPVは迅速に導入できた。本邦の重症心身障害児に対する在宅NPPV導入は、他の呼吸管理法と比べて途上であり、今後の普及が待たれる。(著者抄録)

肢端紅痛症は、四肢末端の発赤・疼痛を特徴とする稀な疾患であるが、その臨床的特徴によって診断するため、同症状を訴える患者を見た際には念頭に置くべき病態である。しばしば難治性で、確立した治療法はない。今回、ステロイドによる早期治療が、慢性疼痛への移行を防ぐうえで有効であったと推測される小児例を経験したので報告する。症例は10歳女児。両手指の色調変化と疼痛を訴えて受診した。手指末端の灼熱痛、色調変化、熱感を認めた。疼痛は加温で増悪し、冷却で軽快した。これら臨床的特徴および他疾患の除外により、肢端紅痛症と診断した。続発性の肢端紅痛症を来す基礎疾患の存在は除外され、一次性肢端紅痛症と考えた。鎮痛薬(アセトアミノフェン)で疼痛は抑えられず、ステロイド薬治療を開始して軽減した。さらに、低用量アスピリンを加え、ステロイド薬の減量を進めながら、ガバペンチンの内服を併用し、疼痛は改善した。(著者抄録)

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.

症例は生来健康な11歳の女児。小学校2年次(7歳時)から不注意症状による学業成績の低下がみられた。8歳以降に体重増加不良、眼球突出、甲状腺腫大が出現し、11歳時にBasedow病と診断された。Basedow病の治療後に不注意症状が著しく改善した経過から、原因不明の学業成績の低下を初期症状とした低年齢の小児の場合には、学習障害や注意欠如多動症(attention deficit hyperactivity disorder:ADHD)のほかに身体疾患であるBasedow病を鑑別に挙げるべきである。(著者抄録)

Autism spectrum disorder (ASD) is characterized by impairment in social communication and the presence of restricted and repetitive behaviors and interests. Executive function impairment is reportedly partially responsible for these symptoms. Executive function includes planning, flexibility, and inhibitory control. Although planning and flexibility in ASD have been consistently reported as atypical, the atypicality of inhibitory control remains controversial. As most previous studies have used nonsocial stimuli to investigate inhibitory control in ASD, the effects of socially relevant information on the inhibitory control system in individuals with ASD remain unclear. Therefore, we developed a go/no-go task with gaze stimuli and measured hemodynamic responses in the right prefrontal cortex (PFC), involved in inhibitory processing in both typically developing (TD) children and children with ASD, using functional near-infrared spectroscopy. Direct gaze induced commission errors to similar extents in both groups. Contrary to the behavioral responses, neural activation in the right PFC was modulated by gaze direction only in the TD group. These findings suggest that the gaze-processing mechanisms in the prefrontal region may be affected by atypical gaze processing in other brain regions during an inhibitory control task with socially relevant information in ASD.