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Evoked potentials in patients with Angelman syndrome

Evoked potentials were studied in four cases with Angelman syndrome. Chromosome 15ql1-13 deletion was proved in two cases and paternal uniparental disomy was proved in the rest. Prolonged photo-evoked eyelid microvibration latencies were noted in all four， while visual evoked potential latencies remained within normal limits in three of four. Interpeak latencies of wave I to wave V in auditory brainstem response were prolonged in two of four. Short latency somatosensory evoked potential was examined in two cases and prolonged interpeak latency of wave P3 to wave N1 was noted in one case. Brink reflex was examined in one case and prolonged R2 latency was noted. These findings suggest that the brainstem is disturbed in cases with Angelman syndrome

Utilization of the Inkling™ for the Rey - Osterrieth Complex Figure test

　The Rey - Osterrieth Complex Figure (ROCF) test assess visual-construction abilility, visual memory and executive function. Three different methods are traditionally used to track and to record the strokes during the production of the complex figure: single pen, switching felt pens and flowcharts. The Inkling™ is an instrument which attributes are to track, to record and to save the information of every stroke depicted in a standard piece of paper. The purpose of the study is to examine the advantages and disadvantages of using the Inkling™ in the ROCF test. 66 ROCF performances were analyzed from subjects aged 6 to 86 years old. Each subject performed the ROCF test twice; first with pen switching method without flowcharts and later with the Inkling™ or viceversa. The comparison of the performances evidenced 5 characteristics: Four subjects distracted by the color of the felt pens, three subjects interfered by the switching of the felt pens, three subjects which performance was facilitated by the colors of the felt pens, nine subjects with unclear score for Fragmentation and fourteen subjects with unclear score for Planning. The advantages that the Inkling™ showed were compensation for the process of flowcharts, avoid interference, and reduce the burden of unclear scoring for Fragmentation and Planning

Evaluation of tritium production rate in a blanket mock-up using a compact fusion neutron source

We report a neutronics study of a blanket mock-up using a discharge-type compact fusion neutron source. Deuterium–deuterium fusion neutrons were irradiated to the mock-ups composed of tritium breeder and neutron reflector/moderator. The tritium production rate (TPR) per source neutron was measured by a single-crystal diamond detector with a 6Li-enriched lithium fluoride film convertor after the calibration process. Despite the low neutron yield, energetic alpha and triton particles via 6Li(n, t)α neutron capture as well as 12C via elastic scattering were successfully detected by the SDD with high signal to noise ratios. The TPRs were experimentally evaluated with errors of 8.4%–8.5% at the 1σ level at the positions with high thermal neutron fluxes where the errors were dominantly introduced by uncertainties in the monitoring of the neutron production rate. The calculated to experimental (C/E) values of TPR were evaluated to be 0.91–1.27 (FENDL-2.1) and 0.94–1.28 (FENDL-3.1). As the neutron source can generate 14 MeV neutrons using a mixed gas of deuterium and tritium, this approach provides more opportunities for blanket neutronics experiments

Developmental Changes in the Trail Making Test

　Trail Making Test（TMT）は，ワーキングメモリや反応抑制，反応の切り替えなどの能力が求められる神経心理学的検査である。TMT は実行機能評価法の一つとして広く用いられているが，子どもを対象とした標準値の作成や臨床応用に関する報告はいまだ少ない。そこで本研究では，TMT の小児期から青年期までの各年齢群別標準値を作成するとともに，各指標の発達的変化について検討した。その結果，Part A の遂行時間，Part B の遂行時間，B－A 値およびB/A 値の４指標において年齢による有意な発達的変化が認められ，本検査は子どもの実行機能の発達の程度を反映しうるものと考えられた。本法は従来のTMT よりも刺激数を削減し簡便化したものであり，注意欠陥／動性障害などの発達障害をともなう子 どもへの臨床応用が期待される

健常児・者におけるVictoria版Stroop Testの年齢による変化の検討

Victoria版Stroop Testにおける課題達成所要時間の年齢による変化について検討し、併せて各年齢群別標準値を得ることを目的とした。5歳1ヶ月から49歳0ヶ月の健常児・者193名を対象に、黒色で印刷された文字の色を読む（Word Reading:WR）課題、丸型カラーバッチの色名を言う（Color Naming:CN）課題、文字と印刷された色が異なる（Incongruent Color Naming:ICN）課題を行い、ICN課題達成所要時間に対するCN課題達成所要時間の差（以下ICN-CN）と比（以下ICN／CN）について年齢を説明変数とする単回帰分析を行った。その結果、全てにおいて年齢による有意な変化が見られ、成績が最良となる年齢は、WR課題において29.7歳、CN課題において29.0歳、ICN課題において30.2歳、ICN-CNにおいて31.0歳、ICN／CNにおいて34.5歳であることが明らかになった。また、各課題達成所要時間の差異についての一要因の分散分析を行った結果、有意差（F（2,576）＝179.312,p<.001）が認められた。多重比較（Turkey HSD)の結果、各課題間に有意差（WR課題とCN課題：p<.01、WR課題とICN課題：p<.001、CN課題とICN課題：p<.001）が認められ、WR課題、CN課題、ICN課題の順に有意な遅延を示した

A case of pulmonary pleomorphic carcinoma with malignant phenotypes induced by ZEB1-associated epithelial-mesenchymal transition

A 60-year-old man was admitted to our hospital with non-small cell lung cancer (NSCLC). Imaging and pathological studies revealed NSCLC, not otherwise specified (NOS), at clinical stage T3N1M0 stage IIIA. We started radiotherapy alone because of obstructive pneumonia and end-stage renal disease, but the tumors progressed rapidly and resulted in death due to air obstruction by pharyngeal metastasis. The cancer was diagnosed as pleomorphic carcinoma in an autopsy. Viable lung tumor cells, which were resistant to radiotherapy, and the pharyngeal metastasis had mesenchymal phenotypes and expressed ZEB1 but not SNAI1. These observations indicated that ZEB1-associated epithelial-mesenchymal transition has malignant features including resistance to radiotherapy and aggressive metastatic potential. ZEB1-associated EMT may be an important mechanism to understand the pathophysiology of pleomorphic carcinoma

Titanium peroxide nanoparticles enhanced cytotoxic effects of X-ray irradiation against pancreatic cancer model through reactive oxygen species generation in vitro and in vivo

Details of the synthetic protocol for PAA-TiOxNPs formation from TiO2NPs. (PPTX 151 kb