关键词： Adaptation   Communication   Needs   Progressive neurology diseases   Quality of life  

摘要： Background Progressive neurological diseases, such as multiple sclerosis, Parkinson's disease, Huntington's disease, significantly interfere with patients' lives, and those of their families. The aim of the research was to establish whether the extent of the information on patients' health conditions, and the way patients learn this information from doctors affect their adaptation to chronic and progressive diseases. Methods Qualitative methodology was used for a total of 52 participants (patients with progressive neurological diseases, their family members, and health and social workers). Data were collected using individual, in-depth interviews and focus groups. Analysis of data for interpretation, conceptualization, and re-integration was performed by open, axial, and selective coding. Results It was determined that adequate information about patients' health status, and the use of coping strategies are related to their adaptation to their disease, and consequently, to their quality of life. The participants often considered the extent of the information provided, and the way they were informed to be inadequate. Receiving the diagnosis, the progression of the disease, and the end of life were found to be the most burdensome. Conclusion Our results show that Czech neurologist should develop better communication skills, particularly for informing patients with progressive neurological diseases. Open communication, emotional support, and support in selecting effective coping strategies can help patients adapt more readily to their disease, and improve their quality of life.

关键词： Traumatic brain injury   NT3(P75-2)   Bone marrow-derived mesenchymal stem cells   Neurological function   P75 signal pathway  

摘要： Background The attainment of extensive neurological function recovery remains the key challenge for the treatment of traumatic brain injury (TBI). Transplantation of bone marrow-derived mesenchymal stem cells (BMSCs) has been shown to improve neurological function recovery after TBI. However, the survival of BMSCs after transplantation in early-stage TBI is limited, and much is unknown about the mechanisms mediating this neurological function recovery. Secretion of neurotrophic factors, including neurotrophin 3 (NT3), is one of the critical factors mediating BMSC neurological function recovery. Gene mutation of NT3 (NT3(P75-2)) has been shown to enhance the biological function of NT3 via the reduction of the activation of the P75 signal pathway. Thus, we investigated whether NT3(P75-2) gene-modified BMSCs could enhance the survival of BMSCs and further improve neurological function recovery after TBI. Methods The ability of NT3(P75-2) induction to improve cell growth rate of NSC-34 and PC12 cells in vitro was first determined. BMSCs were then infected with three different lentiviruses (green fluorescent protein (GFP), GFP-NT3, or GFP-NT3(P75-2)), which stably express GFP, GFP-NT3, or GFP-NT3(P75-2). At 24 h post-TBI induction in mice, GFP-labeled BMSCs were locally transplanted into the lesion site. Immunofluorescence and histopathology were performed at 1, 3, and/or 7 days after transplantation to evaluate the survival of BMSCs as well as the lesion volume. A modified neurological severity scoring system and the rotarod test were chosen to evaluate the functional recovery of the mice. Cell growth rate, glial activation, and signaling pathway analyses were performed to determine the potential mechanisms of NT3(P75-2) in functional recovery after TBI. Results Overall, NT3(P75-2) improved cell growth rate of NSC-34 and PC12 cells in vitro. In addition, NT3(P75-2) significantly improved the survival of transplanted BMSCs and neurological function recovery after TBI. Ov

关键词： Cockayne syndrome rat model   neurodevelopmental disorder   CRISPR/Cas9   transcription dysregulation   CSB  

摘要： Cockayne syndrome (CS) is a rare genetic neurodevelopmental disorder, characterized by a deficiency in transcription-coupled subpathway of nucleotide excision DNA repair (TC-NER). Mutation of the Cockayne syndrome B (CSB) gene affects basal transcription, which is considered a major cause of CS neurologic dysfunction. Here, we generate a rat model by mimicking a nonsense mutation in the CSB gene. In contrast to that of the Csb(-/-) mouse models, the brains of the CSB-deficient rats are more profoundly affected. The cerebellar cortex shows significant atrophy and dysmyelination. Aberrant foliation of the cerebellum and deformed hippocampus are visible. The white matter displays high glial fibrillary acidic protein (GFAP) staining indicative of reactive astrogliosis. RNA sequencing (RNA-seq) analysis reveals that CSB deficiency affects the expression of hundreds of genes, many of which are neuronal genes, suggesting that transcription dysregulation could contribute to the neurologic disease seen in the CSB rat models.

关键词： Long-term disability   Return to work   Cognitive remediation   Psychotherapy   Vocational rehabilitation  

摘要： The current study explores factors predicting return to work in a sample of patients with neurological and neuropsychiatric disorders who have attended a prevocational readiness and social skills training programme many years after trauma. Participants were community-dwelling adults with long-term disabilities (N = 67). Results of univariate analyses followed by multivariate logistic regression analysis revealed that both pre-injury (prior) and post-injury (current) factors influenced the likelihood of employment in our sample: prior employment, current employment readiness, current cognitive competence (particularly memory and executive functioning) and emotional adjustment. Our findings demonstrate that both pre-trauma and current factors interact in predicting return to work not only for individuals with traumatic brain injury (TBI), but also for a broader group of patients with long-term disabilities due to a variety of neurological and neuropsychiatric conditions. Thus, our findings provide preliminary support for ongoing long-term management of individuals with long-term disabilities and warrant close attention of future investigators to potential benefits of cognitive remediation, psychotherapy and vocational rehabilitation in terms of maintenance of initial gains and increased probability of return to work many years after trauma.

关键词： Reasoning skills   Human learning   Reasoning   Taxonomy   Lectures   Statistical distributions   Teachers   Universities  

摘要： Objective To validate a newly-developed Key Feature Problem Examination (KFPE) in neurology, and to examine how it is perceived by students. Methods We have developed a formative KFPE containing 12 key feature problems and 44 key feature items. The key feature problems covered four typical clinical situations. The items were presented in short- and long-menu question formats. Third- and fourth-year medical students undergoing the Neurology Course at our department participated in this study. The students' perception of the KFPE was assessed via a questionnaire. Students also had to pass a summative multiple-choice question examination (MCQE) containing 39 Type-A questions. All key feature and multiple-choice questions were classified using a modified Bloom's taxonomy. Results The results from 81 KFPE participants were analyzed. The average score was 6.7/12 points. Cronbach's alpha for the 12 key-feature problems was 0.53. Item difficulty level scores were between 0.39 and 0.77, and item-total correlations between 0.05 and 0.36. Thirty-two key feature items of the KFPE were categorized as testers of comprehension, application and problem-solving, and 12 questions as testers of knowledge (MCQE: 15 comprehension and 24 knowledge, respectively). Overall correlations between the KFPE and the MCQE were intermediate. The KFPE was perceived well by the students. Conclusions Adherence to previously-established principles enables the creation of a valid KFPE in the field of Neurology.

关键词： Epilepsy   Cognitive function  

摘要： Background Epilepsy is a clinical manifestation of brain disorder due to various etiologies that needs appopriate treatment. Lack of therapy leade to cognitive impairment. This research aims to evaluate cognitive function profile and analyze factors that influence cognitive function in epilepsy *** This is a cross sectional study of epilepsy patient in Mohammad Hoesin Hospital Palembang between January-May 2018 that fullfilled inclusion and exclusion criterias assesed by MOCA-Ina and questionnaire. Data was analyzed with SPSS 20.0 Chi-square, ANOVA and correlation for bivariate and double linear regression for *** Samples consists 39 subjects. The prevalence of cognitive impairment in epilepsy patients was 69.2%. Level of education >9 years was mostly recorded from the samples with MOCA-Ina score that shows cognitive impairment. In multivariate analysis with multiple linear regression tests of 8 variables (age, sex, education level, age of onset, type of epilepsy syndrome, type of drug, duration of seizure, and frequency of seizures), three variables (gender, level of education, duration of seizure) had significant relationship with cognitive function of epilepsy patients. There was also a positive correlation between gender (0,333), education level (0,542) on cognitive function and a negative correlation (−0,334) between the duration of seizure and cognitive *** The prevalence of cognitive impairment was 69.2% in epilepsy patients at Mohammad Hoeasin hospital Palembang. Level of education, gender and duration of seizure influence cognitive function in epilepsy patient.