Three Patients of the Early Onset Epileptic Spasms without Hypsarrhythmia.

Epileptic spasms without hypsarrhythmia occur when patients do not display hypsarrhythmia on electroencephalogram (EEG) at the onset and throughout the clinical course. We report three patients of epileptic spasms in patients with early onset, all of whom experienced other types of seizures.We detail three patients (two boys and one girl) of epileptic spasms without hypsarrhythmia, occurring between 1 and 3 months of age, with no abnormalities detected on neurometabolic analysis and brain magnetic resonance imaging. Long-term video-EEG monitoring revealed epileptic spasms with focal onset seizures in two patients, and epileptic spasms followed by generalized tonic-clonic seizures in one patient. Hypsarrhythmia was never observed in repeated EEG examinations. Two patients achieved seizure freedom and improved development through treatment with topiramate alone or in combination with valproate, without requiring hormonal therapies or vigabatrin. The remaining patient achieved seizure freedom following administration of antiseizure medications, including topiramate, after a trial of adrenocorticotropic hormone therapy.We report the cases of three patients with early onset epileptic spasms without hypsarrhythmia. All patients achieved seizure freedom after topiramate treatment. Topiramate may be considered as a relatively effective antiseizure medication for early onset epileptic spasms without hypsarrhythmia.

An adolescent case of ASXL3-related disorder with delayed onset of feeding difficulty.

BACKGROUND: ASXL3-related disorder, first described in 2013, is a genetic disorder with an autosomal dominant inheritance that is caused by a heterozygous loss-of-function variant in ASXL3. The most characteristic feature is neurodevelopmental delay with consistently limited speech. Feeding difficulty is a main symptom observed in infancy. However, no adolescent case has been reported. CASE PRESENTATION: A 14-year-old girl with ASXL3-related syndrome was referred to our hospital with subacute onset of emotional lability. Limbic encephalitis was ruled out by examination; however, the patient gradually showed a lack of interest in eating, with decreased diet volume. Consequently, she experienced significant weight loss. She experienced no symptoms of bulimia, or food allergy; therefore, avoidant/restrictive food intake disorder (ARFID) was clinically suspected. CONCLUSIONS: We reported the first case of ASXL3-related disorder with adolescent onset of feeding difficulty. ARFID was considered a cause of the feeding difficulty.

Impact of clinical clerkship integrated with clinical ladder on attending physicians' teaching self-efficacy.

BACKGROUND: Self-efficacy plays an important role in enhancing the teaching capabilities of attending physicians (APs). The clinical ladder (CL) is an educational approach developed in the field of nursing education that increases difficulty in an incremental manner. However, no previous study has confirmed the effectiveness of CL in medical education. Therefore, this study aimed to examine the effect of clinical clerkship integrated with clinical ladder (CC-CL) on the self-efficacy of APs. METHODS: Sixth-year medical students participated in CC-CL for 6 months starting from April 2023, and the changes in the self-efficacy of APs were retrospectively evaluated. The students were trained by the APs concurrently, and the achievement levels of each student were shared. The primary outcome measure was the physician teaching self-efficacy questionnaire (PTSQ) score. The PTSQ scores before and after CC-CL were analyzed using the Wilcoxon matched-pair signed-rank test. RESULTS: Fifteen APs from the Department of Pediatric and Child Neurology were included in this study. No significant difference was observed in the total PTSQ scores of the APs before and after CC-CL. However, a significant increase was observed in the PTSQ score of APs who participated for at least 2 h per week over a period of more than 3 months (n = 8) after CC-CL (p = 0.022). Furthermore, APs who had received their pediatrician certification < 10 years ago (n = 8) showed a significant increase in the total PTSQ score after CC-CL (p = 0.022). CONCLUSIONS: CC-CL may play an important role in cultivating the self-efficacy of less experienced APs. Further comparative studies must be conducted in the future to validate the findings of this study.

Photoinduced Phase Transitions of Imine-Based Liquid Crystal Dimers with Twist-Bend Nematic Phases.

Photoisomerizable molecules in liquid crystals (LCs) allow for photoinduced phase transitions, facilitating applications in a wide variety of photoresponsive materials. In contrast to the widely investigated azobenzene structure, research on the photoinduced phase-transition behavior of imine-based LCs is considerably limited. We herein report the thermal and photoinduced phase-transition behaviors of photoisomerizable imine-based LC dimers with twist-bend nematic (NTB) phases. We synthesize two homologous series of ester- and thioether-linked N-(4-cyanobenzylidene)aniline-based bent-shaped LC dimers with an even number of carbon atoms (n = 2, 4, 6, 8, and 10) in the central alkylene spacers, namely, CBCOOnSBA(CN) and CBOCOnSBA(CN), possessing oppositely directed ester linkages, C=OO and OC=O, respectively. Their thermal phase-transition behavior is examined using polarizing optical microscopy and differential scanning calorimetry. All dimers form a monotropic NTB phase below the temperature of the conventional nematic (N) phase upon cooling. Remarkably, the NTB phases of CBCOOnSBA(CN) (n = 2, 4, 6, and 8) and CBOCOnSBA(CN) (n = 6 and 8) supercool to room temperature and vitrify without crystallization. In addition, the phase-transition temperatures and entropy changes of CBCOOnSBA(CN) are lower than those of CBOCOnSBA(CN) at the same n. Under UV light irradiation, the NTB and N phases transition to the N and isotropic phases, respectively, and reversibly return to their initial LC phases when the UV light is turned off.

The Impact of Simulation-Based Training on Self-Efficacy in Sedation for Pediatric Magnetic Resonance Imaging.

Background: In Japan, approximately 35% of facilities experience sedation-related complications for pediatric magnetic resonance imaging (MRI), including severe complications such as respiratory and cardiac arrests. In the medical education field, simulation improves not only the quality of real emergency response but also health care workers' self-efficacy. Individuals with high self-efficacy are better prepared at handling diverse conditions. However, there is no research examining the impact of sedation simulation for pediatric MRI on the self-efficacy of health care workers. This study aimed to retrospectively investigate the impact of sedation simulation for pediatric MRI on the self-efficacy of health care workers in sedation. Methods: The study was conducted on August 18, 2023, and enrolled pediatricians, nurses, and radiological technologists. The impact of sedation simulation for pediatric MRI on the participants was analyzed using confidence scale scores and part of the Pediatric Resuscitation and Escalation of Care Self-Efficacy Scale (PRSES), before and after sedation simulation for pediatric MRI. Results: Eighteen participants (six pediatricians, six nurses, and six radiological technologists) were included in this study. Regarding confidence scale scores, a significant improvement was observed in the overall group (P = 0.002) and among the nurses (P = 0.0036). Regarding the item 'When confronted with a clinically deteriorating child, I know how to ask for assistance' of PRSES, a significant improvement was observed in the overall group (P = 0.0035) and among the radiological technologists (P = 0.048). Conclusion: There's a potential for sedation simulation for pediatric MRI to increase the self-efficacy of health care workers in MRI sedation. Our findings suggest that this training has a valuable role in preparing health care workers to practice sedation for pediatric MRI.

Assessing the Subjective Happiness of Parents of Children With Severe Motor and Intellectual Disabilities Receiving Home Care.

BACKGROUND: Home care for children with severe motor and intellectual disabilities (SMID) is challenging for parents because it is highly intensive and long-lasting. The pursuit of happiness is an essential goal for everyone. However, only a few studies have focused on the happiness of families with such children. OBJECTIVE: The study aimed to examine the subjective happiness of parents of children with SMID receiving home care and identify the factors associated with their happiness. METHODS: We conducted a cross-sectional online questionnaire-based survey of 23 parents of children with SMID and nurses with children without disabilities as controls at Tottori University Hospital, Yonago, Japan from July 1 to August 31, 2023. We set the subjective happiness scale (SHS) scores as the outcomes. We used the Mann-Whitney U test to compare the SHS scores between the two groups. Moreover, we extracted the clinical and demographic factors affecting the SHS scores of parents of children with SMID using univariate linear regression analysis. RESULTS: We obtained responses from 12 parents with SMID and 105 controls. The average SHS scores of parents with SMID and controls were 4.8 and 4.7, respectively, and both groups did not differ significantly. Univariate analysis showed that parental male sex and the presence of a tracheostomy were negatively associated with the SHS scores of parents. CONCLUSIONS: The SHS scores did not differ significantly between parents with SMID and controls. However, more attention seemed necessary for fathers and parents of children who have undergone tracheostomies. Given the exploratory nature of this study and its small sample size, larger-scale investigations are warranted. Additionally, qualitative research conducted after establishing trustful relationships could provide further insights.

Successful perioperative preparation of a child with autism spectrum disorder in collaboration with his school for special needs education: a case report.

Introduction: The incidence of autism spectrum disorder (ASD) in children is greater than 1%. Children with ASD show an increased rate of hospital contact for several reasons. Perioperative preparation for children with ASD can be challenging; therefore, obtaining information from patients' families prior to surgery is important. However, no previous reports have described the collection of information from educational facilities. Case report: A 12 years-old male patient with ASD was referred for surgery for traumatic dislocation of the left knee joint. Before admission, we obtained valuable information from his parents regarding expected behavioral problems and coping strategies during hospitalization and from his teachers at his school for special needs education. In particular, the information obtained from teachers was specific and practical. Consequently, we could effectively conduct perioperative management based on his specific autistic characteristics. Conclusion: We report a pediatric case of ASD in which favorable perioperative management was successfully achieved by collecting information before admission from family members as well as teachers at the patient's school for special needs education. This management may help in future hospital admissions for children with autism.