Altered empathy processing in frontotemporal dementia A task-based fMRI study.

A lack of empathy, and particularly its affective components, is a core symptom of behavioural variant frontotemporal dementia (bvFTD). Visual exposure to images of a needle pricking a hand (pain condition) and Q-tips touching a hand (control condition) is an established functional magnetic resonance imaging (fMRI) paradigm used to investigate empathy for pain (EFP; pain condition minus control condition). EFP has been associated with increased blood oxygen level dependent (BOLD) signal in regions known to become atrophic in the early stages in bvFTD, including the anterior insula and the anterior cingulate. We therefore hypothesized that patients with bvFTD would display altered empathy processing in the EFP paradigm. Here we examined empathy processing using the EFP paradigm in 28 patients with bvFTD and 28 sex and age matched controls. Participants underwent structural MRI, task-based and resting-state fMRI. The Interpersonal Reactivity Index (IRI) was used as a measure of different facets of empathic function outside the scanner. The EFP paradigm was analysed at a whole brain level and using two regions-of-interest approaches, one based on a metanalysis of affective perceptual empathy versus cognitive evaluative empathy and one based on the controls activation pattern. In controls, EFP was linked to an expected increase of BOLD signal that displayed an overlap with the pattern of atrophy in the bvFTD patients (insula and anterior cingulate). Additional regions with increased signal were the supramarginal gyrus and the occipital cortex. These latter regions were the only ones that displayed increased BOLD signal in bvFTD patients. BOLD signal increase under the affective perceptual empathy but not the cognitive evaluative empathy region of interest was significantly greater in controls than in bvFTD patients. The controls rating on their empathic concern subscale of the IRI was significantly correlated with the BOLD signal in the EFP paradigm, as were an informants ratings of the patients empathic concern subscale. This correlation was not observed on other subscales of the IRI or when using the patient's self-ratings. Finally, controls and patients showed different connectivity patterns in empathy related networks during resting-state fMRI, mainly in nodes overlapping the ventral attention network. Our results indicate that reduced neural activity in regions typically affected by pathology in bvFTD is associated with reduced empathy processing, and a predictor of patients capacity to experience affective empathy.

Elevating pediatric urology care: The crucial role of nursing research in quality improvement.

This educational article highlights the critical role of pediatric urology nursing research in improving the care of children with urological conditions. It discusses the multifaceted nature of pediatric urology nursing, addresses challenges such as limited nurse scientists and resource constraints, and highlights the need to overcome barriers to increase research involvement. The authors emphasize the importance of prioritizing research areas, the promotion of collaboration, and the provision of adequate funding and academic time for pediatric nurses to contribute to evidence-based practice, to improve patient outcomes. Furthermore, it highlights the importance of research in advancing nursing practice, shaping protocols, and advocating for the rights and needs of children with urological conditions and their families.

The importance of animal specificity in animal experimentation, part I: Anatomy in relation to pediatric urology.

Why and when is animal experimentation relevant? The answer to this question depends on the research question. In this short educational article we aim to raise awareness of the importance of formulating a very specific research question before choosing an animal species. An awareness of anatomical and physiological differences vis-a-vis similarities between species, will increase the potential for obtaining data that is relevant for translation to human conditions.

The importance of animal specificity in animal experimentation, part II: Physiological challenges and opportunities in relation to pediatric urology.

When performing animal experimentation in Pediatric Urology studies, it is important to be aware of physiological differences between species and to understand when relevant disease models are available. Diseased animal models may be more relevant in many cases, rather than performing studies in healthy and normally developed animals. For example, they may be more appropriate for the study of congenital malformations, to investigate the secondary effects of prenatal urinary obstruction, to study the effect of prenatal exposure to endogenous or exogenous factors which may lead to disease, or in testing bioengineered structures. In this short educational article, we aim to describe some disease models that have been used to simulate human pathologies and how, if properly designed, these studies can lead to important new knowledge for human translation. In addition, we also highlight the importance of formulating a research question(s) before deciding on the animal experimental model and species to choose.

Structural and functional connectivity associations with anterior cingulate sulcal variability.

Background: Sulcation of the anterior cingulate may be defined by presence of a paracingulate sulcus, a tertiary sulcus developing during the third gestational trimester with implications on cognitive function and disease. Methods: In this retrospective analysis we examine task-free resting state functional connectivity and diffusion-weighted tract segmentation data from a cohort of healthy adults (< 60-year-old, n = 129), exploring the impact of ipsilateral paracingulate sulcal presence on structural and functional connectivity. Results: Presence of a left paracingulate sulcus was associated with reduced fractional anisotropy in the left cingulum (P = 0.02) bundle and the peri-genual (P = 0.002) and dorsal (P = 0.03) but not the temporal cingulum bundle segments. Left paracingulate sulcal presence was associated with increased left peri-genual radial diffusivity (P = 0.003) and tract volume (P = 0.012). A significant, predominantly intraregional frontal component of altered resting state functional connectivity was identified in individuals possessing a left PCS (P = 0.01). Seed-based functional connectivity in pre-defined networks was not associated with paracingulate sulcal presence. Conclusion: These results identify a novel association between neurodevelopmentally derived sulcation and altered structural connectivity in a healthy adult population with implications for conditions where this variation is of interest. Furthermore, they provide evidence of a link between the structural and functional connectivity of the brain in the presence of a paracingulate sulcus which may be mediated by a highly connected local functional network reliant on short association fibres.

Validation of the French version of the Caregivers' Priorities and Child Health Index of Life with Disabilities questionnaire.

INTRODUCTION: The Caregivers' Priorities and Child Health Index of Life with Disabilities (CPCHILD) is a questionnaire that measures the health-related quality of life (HRQL) of children with cerebral palsy (CP). Though measuring HRQL is challenging in these children, it is a valuable help for medical decision-making. There is no questionnaire to assess HRQL in French-speaking children with severe CP. OBJECTIVE: To translate and adapt transculturally the CPCHILD questionnaire into French (CPCHILD-FV). MATERIAL AND METHODS: The CPCHILD was translated from English into French by forward and backward translation by independents translators. The questionnaire was then tested on 32 caregivers of patients with CP classified as GMFCS IV or V, remarks of caregivers were analyzed by an expert committee and, if necessary, modifications were performed. Internal consistency of the CPCHILD-FV was assessed using a sample of 32 parents or caregivers and test-retest reliability was assessed on a random sample of 10 patients. RESULTS: The translation and transcultural process resulted in a French version of the CPCHILD. Some items of the CPCHILD required careful discussion to ensure that items had the same meaning as in the original. Internal consistencies were over 0.70 for each domain except for health, and 0.97 for the total scores. The ICC for the test-retest reliability of the CHILD-FV total score was 0.98 (95% CI: 0.93-0.99) and ranged from 0.59 to 0.99 for the domains. CONCLUSION: The translation and cross-cultural adaptation of the CPCHILD questionnaire provides a French version than can measure the HRQL of children with severe CP. LEVEL OF EVIDENCE: IV; prospective study without control group.

Anterior cingulate sulcation is associated with onset and survival in frontotemporal dementia.

Frontotemporal dementia is the second most common form of early onset dementia (<65 years). Despite this, there are few known disease-modifying factors. The anterior cingulate is a focal point of pathology in behavioural variant frontotemporal dementia. Sulcation of the anterior cingulate is denoted by the presence of a paracingulate sulcus, a tertiary sulcus developing, where present during the third gestational trimester and remaining stable throughout life. This study aims to examine the impact of right paracingulate sulcal presence on the expression and prognosis of behavioural variant frontotemporal dementia. This retrospective analysis drew its population from two clinical samples recruited from memory clinics at university hospitals in the USA and The Netherlands. Individuals with sporadic behavioural variant frontotemporal dementia were enrolled between 2000 and 2022 and followed up for an average of 7.71 years. T1-MRI data were evaluated for hemispheric paracingulate sulcal presence in accordance with an established protocol by two blinded raters. Outcome measures included age at onset, survival, cortical thickness and Frontotemporal Lobar Degeneration-modified Clinical Dementia Rating determined clinical disease progression. The study population consisted of 186 individuals with sporadic behavioural variant frontotemporal dementia (113 males and 73 females), mean age 63.28 years (SD 8.32). The mean age at onset was 2.44 years later in individuals possessing a right paracingulate sulcus [60.2 years (8.54)] versus individuals who did not [57.76 (8.05)], 95% confidence interval > 0.41, P = 0.02. Education was not associated with age at onset (ß = -0.05, P = 0.75). The presence of a right paracingulate sulcus was associated with an 83% increased risk of death per year after age at onset (hazard ratio 1.83, confidence interval [1.09-3.07], P < 0.02), whilst the mean age at death was similar for individuals with a present and absent right paracingulate sulcus (P = 0.7). Right paracingulate sulcal presence was not associated with baseline cortical thickness. Right paracingulate sulcal presence is associated with disease expression and survival in sporadic behavioural variant frontotemporal dementia. Findings provide evidence of neurodevelopmental brain reserve in behavioural variant frontotemporal dementia that may be important in the design of trials for future therapeutic approaches.

Risk of repeated drainage in pediatric septic arthritis: patient or method?

How drainage of septic arthritis should be performed remains controversial. The aim of the present study was to compare arthrocentesis (Ac) using double intra-articular needle lavage to arthrotomy (At) as first-line drainage treatment for pediatric hip and knee septic arthritis. The secondary objective was to identify risk factors of second articular drainage. A retrospective review of medical records of children with knee and hip septic arthritis was conducted. Inclusion criteria were: children treated for septic arthritis between 2014 and 2020 with a positive culture of joint fluid. Clinical, biological, radiographical and ultrasound data were recorded at presentation and during follow-up. Patients were divided into 2 groups according to the type of drainage performed: Ac or At. 25 hips and 44 knees were included, 42 treated by Ac (15 hips, 27 knees) and 27 by At (10 hips, 17 knees). There is no significant difference between Ac and At regarding the need for repeated drainage and Ac nor At was reported as risk factor for repeated drainage. The presence of associated musculoskeletal infection (MSI) was a significant risk factor of repeated drainage [odds ratio = 11.8; 95% confidence interval = 1.2-114.2; P < 0.001]. Significantly more associated MSI (P < 0.001), level I virulence germs (P < 0.001) and positive blood culture (<0.001) were found in patients who underwent repeated drainage. There was no significant difference between Ac and At regarding rate of repeated drainage. The risk factors for repeated drainage were: associated with MSI, virulent germs and positive blood culture.

Translation and validation of the French version of the adolescent idiopathic scoliosis Truncal Anterior Asymmetry Scoliosis Questionnaire (TAASQ).

PURPOSE: Scoliosis is an abnormality which causes anterior trunk asymmetry. The Truncal Anterior Asymmetry Scoliosis Questionnaire-adolescent idiopathic scoliosis (AIS) (TAASQ) measures the feeling about anterior trunk appearance in girls with AIS. It comprises 14 questions which evaluate frontal asymmetry of breasts, shoulders, and waist as well as behavioral attitude. There is currently no validated questionnaire in French to evaluate truncal asymmetry in AIS girls. The aim of this study was to translate and validate the French version of the TAASQ (TAASQ-VF). METHODS: The study reports the translation and transcultural adaptation of the TAASQ into French. The translation was tested on 20 patients to verify comprehension and modify if necessary. To assess the reliability of the translated version, each domain as well as the total measure were tested for internal consistency. Convergent validity was evaluated on 63 patients and test-retest on a sample of 15. RESULTS: The translation and content validation process resulted in a French version of the TAASQ. Internal consistency was over 0.80 for each item, over 0.70 for each domain, and 0.88 for the total scores. SAQ were used to perform convergent validity with TAASQ-VF. The TAASQ-FV correlated well with many of the SAQ domains (p < 0.05) and every TAASQ-FV domain correlated with at least one SAQ domain (p < 0.05). Test-retest reliability for the total score and for each domain was good. CONCLUSION: The translation and cross-cultural adaptation of the TAASQ questionnaire provides a French version that can measure the feeling about frontal appearance in girls with idiopathic scoliosis.

Financial and relational impact of having a boy with posterior urethral valves.

Introduction: Childhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care. Patients and method: We gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards. The questionnaire included questions about the parents' demographics, health, professional, financial and marital status and how these evolved since the child's birth as well as the "impact on family scale" (IOFS), which gives a total score ranging from 15 (no impact) to 60 (maximum impact). We then analyzed both the results of the specific demographic questions as well as the factors which influenced the IOFS score. Results: We retrieved answers for 38/51 families (74.5% response rate). The average IOFS score was 23.7 (15-51). We observed that the child's creatinine level had an effect on the IOFS score (p = 0.02), as did the parent's gender (p = 0.008), health status (p = 0.015), being limited in activity since the birth of the child (p = 0.020), being penalized in one's job (p = 0.009), being supported in one's job (p = 0.002), and decreased income (p = 0.004). Out of 38 mother/father binomials, 8/33 (24.2%) declared that they were no longer in the same relationship afterwards. Conclusion: In conclusion, having a boy with PUV significantly impacts families. The risk of parental separation and decrease in revenue is significant. Strategies aiming to decrease these factors should be put in place as soon as possible.

Intraoperative neurophysiological monitoring in scoliosis surgery: literature review of the last 10 years.

INTRODUCTION: Patients with spinal deformities undergoing corrective surgery are at risk for iatrogenic spinal cord injury (SCI) and subsequent neurological deficit. Intraoperative neurophysiological monitoring (IONM) allows early detection of SCI which enables early intervention resulting in a better prognosis. The primary aim of this literature review was to search if there are threshold values of TcMEP and SSEP in the literature that are widely accepted as alert during IONM. The secondary aim was to update knowledge concerning IONM during scoliosis surgery. METHOD: PubMed/MEDLINE and Cochrane library electronic databases were used to search publication from 2012 to 2022. The following keywords were used: evoked potential, scoliosis, surgery, intraoperative monitoring and neurophysiological. We included all studies dealing with SSEP and TcMEP monitoring during scoliosis surgery. Two authors reviewed all titles and abstracts to identify studies that met the inclusion criteria. RESULTS: We included 43 papers. Rates of IONM alert and neurological deficit varied from 0.56 to 64% and from 0.15 to 8.3%, respectively. Threshold values varied from a loss of 50 to 90% for TcMEP amplitude, whereas it seems that a loss of 50% in amplitude and/or an increase of 10% of latency is widely accepted for SSEP. Causes of IONM changes most frequently reported were surgical maneuver. CONCLUSION: Concerning SSEP, a loss of 50% in amplitude and/or an increase of 10% of latency is widely accepted as an alert. For TcMEP, it seems that the use of highest threshold values can avoid unnecessary surgical procedure for the patient without increasing risk of neurological deficit.

Anterior cingulate sulcation is associated with onset and survival in frontotemporal dementia.

Background: Frontotemporal dementia is the second most common form of early onset dementia (< 65 years). Despite this there are few known disease modifying factors. The anterior cingulate is a focal point of pathology in behavioural variant frontotemporal dementia. Sulcation of the anterior cingulate is denoted by the presence of a paracingulate sulcus, a tertiary sulcus developing, where present during the third gestational trimester and remaining stable throughout life. This study aims to examine the impact of right paracingulate sulcal presence on the expression and prognosis of behavioural variant Frontotemporal Dementia. Methods: This retrospective analysis drew it's population from two clinical samples recruited from memory clinics at University Hospitals in The United States of America and The Netherlands. Individuals with sporadic behavioural variant Frontotemporal Dementia were enrolled between 2004 and 2022 and followed up for an average of 7.71 years. T1-MRI data were evaluated for hemispheric paracingulate sulcal presence in accordance with an established protocol by two blinded raters. Outcome measures included age at onset, survival, cortical thickness, and Frontotemporal Lobar Degeneration-modified Clinical Dementia Rating determined clinical disease progression. Results: The study population consisted of 186 individuals with sporadic behavioural variant Frontotemporal Dementia, (113 males and 73 females) mean age 63.28 years (SD 8.32). The mean age at onset was 2.44 years later in individuals possessing a right paracingulate sulcus (60.2 years (SD 8.54)) versus individuals who did not (57.76 (8.05)), 95% CI >0.41, P = 0.02. Education was not associated with age at onset (ß = -0.05, P =0.75). Presence of a right paracingulate sulcus was associated with a 119% increased risk of death per year after age at onset (HR 2.19, CI [1.21 - 3.96], P <0.01), whilst the mean age at death was similar for individuals with a present and absent right paracingulate sulcus ( P = 0.7). Right paracingulate sulcal presence was not associated with baseline cortical thickness. Conclusion: Right paracingulate sulcal presence is associated with disease expression and survival in sporadic behavioural variant Frontotemporal Dementia. Findings provide evidence of neurodevelopmental brain reserve in behavioural variant Frontotemporal Dementia which may be important in the design of trials for future therapeutic approaches.

Long-term results of first row carpectomy with proximal capitate resurfacing using a pyrocarbon implant.

The functional results of proximal row carpectomy with a proximal capitate resurfacing using a pyrocarbon implant were evaluated in a retrospective, multicentric, multi-operator study. The outcomes of patients operated on at five surgical centres between January 2005 and December 2018 were reviewed. The data were collected by an independent observer during standardized consultations. Based on 30 patients with a mean follow-up of 7 years, the median range of flexion and extension was 65° and the median radioulnar tilt was 45°. The median Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH) score was 14 and the improvements in strength and pain were good. Compared with the preoperative values, significant improvements were seen in the flexion and extension range of motion, radioulnar tilt and pain score. No postoperative complications were recorded.Level of evidence: IV.

[18F]RO948 tau positron emission tomography in genetic and sporadic frontotemporal dementia syndromes.

PURPOSE: To examine [18F]RO948 retention in FTD, sampling the underlying protein pathology heterogeneity. METHODS: A total of 61 individuals with FTD (n = 35), matched cases of AD (n = 13) and Aß-negative cognitively unimpaired individuals (n = 13) underwent [18F]RO948PET and MRI. FTD included 21 behavioral variant FTD (bvFTD) cases, 11 symptomatic C9orf72 mutation carriers, one patient with non-genetic bvFTD-ALS, one individual with bvFTD due to a GRN mutation, and one due to a MAPT mutation (R406W). Tracer retention was examined using a region-of-interest and voxel-wise approaches. Two individuals (bvFTD due to C9orf72) underwent postmortem neuropathological examination. Tracer binding was additionally assessed in vitro using [3H]RO948 autoradiography in six separate cases. RESULTS: [18F]RO948 retention across ROIs was clearly lower than in AD and comparable to that in Aß-negative cognitively unimpaired individuals. Only minor loci of tracer retention were seen in bvFTD; these did not overlap with the observed cortical atrophy in the cases, the expected pattern of atrophy, nor the expected or verified protein pathology distribution. Autoradiography analyses showed no specific [3H]RO948 binding. The R406W MAPT mutation carriers were clear exceptions with AD-like retention levels and specific in-vitro binding. CONCLUSION: [18F]RO948 uptake is not significantly increased in the majority of FTD patients, with a clear exception being specific MAPT mutations.