Agreement Between House-Brackmann and Sunnybrook Facial Nerve Grading Systems in Bell's Palsy in Children: Secondary Analysis of a Randomized, Placebo-Controlled Multicenter Trial.

Background: There is limited evidence on the use of facial nerve function grading scales in acute facial nerve paralysis in children.

Objective: To investigate the agreement between and the usability of the House-Brackmann and Sunnybrook scales in children with idiopathic facial paralysis (Bell's palsy) and to compare their ease of administration.

Methods: Data from a randomized controlled trial in children aged 6 months to <18 years with Bell's palsy was used.

Visualising Patterns in Women's Experiences of Intimate Partner Violence in the First 10 years of Motherhood.

Intimate partner violence (IPV) can involve patterns of physical, sexual and emotional abuse. Women typically experience physical IPV in combination with emotional IPV, while emotional IPV is often experienced in the absence of other types of IPV. There is very little known about women's experiences of these different types of IPV over time.

Parenteral Long-Acting Antiseizure Medications Are Used More Often to Treat Seizure Clusters Than Convulsive Status Epilepticus in the Pediatric Emergency Department.

Recent trials provide high-quality evidence for second-line treatment of convulsive status epilepticus (CSE) in children. However, the most effective medications for other seizure emergencies are poorly understood without established treatment algorithms. We investigated children presenting to the emergency department with repetitive or prolonged convulsions who required intravenous long-acting antiseizure medications, to determine the relative importance and treatment responsiveness of status epilepticus and seizure clusters.

Competing risks analysis with missing cause-of-failure-penalized likelihood estimation of cause-specific Cox models.

Competing risks models are attractive tools to analyze time-to-event data where several causes of an event are competing. However, a complexity may arise when, for instance, some subjects experience the event of interest but the causes are not known. Assuming that unknown causes of events are missing at random, we developed a novel constrained maximum penalized likelihood method for fitting semi-parametric cause-specific Cox regression models.

Reproduction and Genetic Responsibility: An Interpretive Description of Reproductive Decision-Making for Young People With Li-Fraumeni Syndrome.

The reproductive decision-making of young people (aged 15-39 years) with Li-Fraumeni syndrome (LFS), an early onset inherited cancer syndrome, has not been studied in depth. Using interpretive description methodology, we conducted semi-structured interviews with 30 young Australians (mean age 25.5 years) diagnosed with LFS or at 50% genetic risk.

Digital Mega-Studies as a New Research Paradigm: Governing the Health Research of the Future.

'Digital Mega-Studies' are entirely or extensively digitised, longitudinal, population-scale initiatives, collecting, storing, and making available individual-level research data of different types and from multiple sources, shaped by technological developments and unforeseeable risks over time. The Australian 'Gen V' project exemplifies this new research paradigm. In 2019, we undertook a multidisciplinary, multi-stakeholder process to map Digital Mega-Studies' key characteristics, legal and governance challenges and likely solutions.

Preventing internalising problems in preschoolers with chronic physical health conditions.

This pilot study aimed to explore the suitability of a preventative intervention for internalising problems in young children with chronic physical health conditions. The pilot study focused on a subsample of 27 children with chronic physical health conditions within a population-level randomised controlled trial of the Cool Little Kids parenting programme. The Cool Little Kids parenting programme aims to prevent the development of internalising problems in inhibited (shy/anxious) preschool children by educating parents to reduce young children's avoidant coping styles and manage their anxiety/distress.

Validation of a measure of parental responsiveness: Comparison of the brief Parental Responsiveness Rating Scale with a detailed measure of responsive parental behaviours.

Parental responsiveness is vital for child language development. Its accurate measurement in clinical settings could identify families who may benefit from preventative interventions; however, coding of responsiveness is time-consuming and expensive. This study investigates in a clinical context the validity of the Parental Responsiveness Rating Scale (PaRRiS): a time- and cost-effective global rating scale of parental responsiveness.

The General Movements Assessment in Neonates With Hypoxic Ischemic Encephalopathy.

Background: Clinical measures after birth and studies such as electroencephalogram (EEG) and brain imaging do not fully predict neurodevelopmental outcomes of infants with hypoxic-ischemic encephalopathy. Early detection of adverse neurologic outcomes, and cerebral palsy in particular, in high-risk infants is essential for ensuring timely management. The General Movements Assessment is a tool that can be used in the early detection of cerebral palsy in infants with brain injury.

Chudley-McCullough Syndrome: A Recognizable Clinical Entity Characterized by Deafness and Typical Brain Malformations.

Chudley-McCullough syndrome, a rare autosomal recessive disorder due to pathogenic variants in the (G-protein signaling modulator 2) gene, is characterized by early-onset sensorineural deafness and a typical combination of brain malformations, including ventriculomegaly, (partial) agenesis of the corpus callosum, cerebellar dysplasia, arachnoid cysts, frontal subcortical heterotopia, and midline polymicrogyria. When hearing loss is managed early, most patients have minor or no impairment of motor and cognitive development, despite the presence of brain malformations. We report 2 cases of Chudley-McCullough syndrome, one presenting with congenital deafness and normal development except for speech delay and one presenting prenatally with ventriculomegaly and an atypical postnatal course characterized by epileptic spasms, deafness, and moderate intellectual disability.

Cerebral Microangiopathy in Leukoencephalopathy With Cerebral Calcifications and Cysts: A Pathological Description.

Leukoencephalopathy with calcifications and cysts (LCC) is a neurological syndrome recently associated with pathogenic variants in . We report autopsy neuropathological findings from an individual with genetically confirmed LCC. Histologic studies included staining of formalin-fixed paraffin-embedded tissue sections by hematoxylin and eosin, elastic van Gieson, and luxol fast blue.