Challenges in Using Parent-Reported Bed and Wake Times for Actigraphy Scoring in Rett-Related Syndromes.

Sleep problems are common in Rett syndrome and other neurogenetic syndromes. Actigraphy is a cost-effective, objective method for measuring sleep. Current guidelines require caregiver-reported bed and wake times to facilitate actigraphy data scoring.

International workshop: what is needed to ensure outcome measures for Rett syndrome are fit-for-purpose for clinical trials? June 7, 2023, Nashville, USA.

Introduction: The clinical, research and advocacy communities for Rett syndrome are striving to achieve clinical trial readiness, including having fit-for-purpose clinical outcome assessments. This study aimed to (1) describe psychometric properties of clinical outcome assessment for Rett syndrome and (2) identify what is needed to ensure that fit-for-purpose clinical outcome assessments are available for clinical trials.

Methods: Clinical outcome assessments for the top 10 priority domains identified in the Voice of the Patient Report for Rett syndrome were compiled and available psychometric data were extracted.

Travis I. Thompson (1937-2023).

Travis I. Thompson was born on July 20, 1937, in Minneapolis to William and Loretta Thompson. Travis completed his BA (1958), MA (1961), and PhD (1961) degrees all at the University of Minnesota in Psychology, with postdoctoral work at the University of Maryland with Joseph V.

Self and caregiver report measurement of sensory features in autism spectrum disorder: a systematic review of psychometric properties.

Background: Unusual responses to sensory stimuli are considered a diagnostic symptom of autism spectrum disorder with mounting research efforts put towards understanding, characterizing, and treating such symptoms.

Methods: This paper examines self and caregiver report tools used to measure sensory features in ASD through a systematic review of the psychometric evidence for their use. A total of 31 empirical papers were reviewed across 20 assessment tools.

Modifying quantitative sensory testing to investigate behavioral reactivity in a pediatric global developmental delay sample: Relation to peripheral innervation and chronic pain outcomes.

Early tactile and nociceptive (pain) mechanisms in children with global developmental delay at risk for intellectual and developmental disability are not well understood. Sixteen children with global developmental delay (mean age = 5.1 years, SD = 1.

A systematic review of risk for the development and persistence of self-injurious behavior in intellectual and developmental disabilities.

Self-injurious behavior (SIB) by individuals with intellectual and developmental disabilities including autism (I/DD) is among the most clinically disturbing, socially costly, and scientifically challenging behavior disorders. Forty years of clinical research has produced a knowledge base supporting idiographic behavioral assessment and treatment approaches. Despite the treatment progress, from a public health and population perspective, we argue it is less clear that we have reduced the disorder's burden.

Pain burden in children with cerebral palsy (CPPain) survey: Study protocol.

Pain is a significant health concern for children living with cerebral palsy (CP). There are no population-level or large-scale multi-national datasets using common measures characterizing pain experience and interference (ie, pain burden) and management practices for children with CP. The aim of the CPPain survey is to generate a comprehensive understanding of pain burden and current management of pain to change clinical practice in CP.

Presence and predictors of pain after orthopedic surgery and associated orthopedic outcomes in children with cerebral palsy.

While children with cerebral palsy (CP) may undergo 8-22 orthopedic surgeries in their lifetime, little is known about the associated pain. We aimed to assess the pain presence before and one year after lower extremity orthopedic surgery, predictors of pain presence at follow-up, and the association between pain and orthopedic outcomes related to surgery. This retrospective study included 86 children with CP (M age = 10.

Parental Pain Catastrophizing, Communication Ability, and Post-surgical Pain Outcomes Following Intrathecal Baclofen Implant Surgery for Patients With Cerebral Palsy.

There is strong evidence that psychosocial variables, including pain catastrophizing, influence parental and child ratings of pain, pain expression, and long-term outcomes among children with chronic pain. The role of these factors among children who have communication deficits due to cerebral palsy (CP) and other intellectual and developmental disabilities is currently unclear. In this study, parental pain catastrophizing was assessed before intrathecal baclofen (ITB) pump implantation for spasticity management in 40 children and adolescents with CP, aged 4 to 24 years.

Expression of and correlational patterns among neuroinflammatory, neuropeptide, and neuroendocrine molecules from cerebrospinal fluid in cerebral palsy.

Background: The underlying pathogenesis of cerebral palsy (CP) remains poorly understood. The possibility of an early inflammatory response after acute insult is of increasing interest. Patterns of inflammatory and related biomarkers are emerging as potential early diagnostic markers for understanding the etiologic diversity of CP.

[Challenges in pain assessment and management among individuals with intellectual and developmental disabilities : German version].

Introduction: Intellectual and developmental disabilities (IDD) include conditions associated with physical, learning, language, behavioural, and/or intellectual impairment. Pain is a common and debilitating secondary condition compromising functional abilities and quality of life.

Objectives: This article addresses scientific and clinical challenges in pain assessment and management in individuals with severe IDD.

Caregiver-Reported Pain Management Practices for Individuals With Cerebral Palsy.

Objective: To investigate types and intensity of pain experienced by individuals with cerebral palsy (CP) and common pain-relieving approaches used by caregivers.

Design: The approach was cross-sectional, using standardized interviews.

Setting: Individuals with CP were recruited from a specialty health care hospital.

Musculoskeletal Pain Outcomes Pre- and Post Intrathecal Baclofen Pump Implant in Children With Cerebral Palsy: A Prospective Cohort Study.

Objective: To characterize musculoskeletal pain intensity, duration, frequency, and interference with activities of daily living in children with cerebral palsy (CP) before and after intrathecal baclofen pump placement.

Design: Prospective cohort study.

Setting: Children's tertiary hospital.

Associations Among Diurnal Salivary Cortisol Patterns, Medication Use, and Behavioral Phenotype Features in a Community Sample of Rett Syndrome.

Rett syndrome (RTT) is a severe neurodevelopmental disorder resulting from mutations of the MECP2 gene. Hyperactivity of the hypothalamic-pituitary-adrenal (HPA) axis and abnormal stress responses have been observed in animal models of RTT, but little is known about HPA axis function among individuals with RTT. Diurnal salivary cortisol patterns from 30 females with RTT were examined in relation to mutation type, medication use, and features of the RTT behavioral phenotype.

Challenges in pain assessment and management among individuals with intellectual and developmental disabilities.

Introduction: Intellectual and developmental disabilities (IDD) include conditions associated with physical, learning, language, behavioural, and/or intellectual impairment. Pain is a common and debilitating secondary condition compromising functional abilities and quality of life.

Objectives: This article addresses scientific and clinical challenges in pain assessment and management in individuals with severe IDD.

Delay to Early Intensive Behavioral Intervention and Educational Outcomes for a Medicaid-Enrolled Cohort of Children with Autism.

Increased prevalence of autism spectrum disorder (ASD) has underscored the need for early intervention services. Early Intensive Behavioral Intervention (EIBI) is among the most common evidence-based approaches, however, stakeholders report significant waitlists. The effects of these delays to intervention are unknown.

Special Education Supports and Services for Rett Syndrome: Parent Perceptions and Satisfaction.

There are no published studies describing educational experiences for girls with Rett syndrome. Given the extensive educational needs associated with Rett syndrome, it is important to understand how families perceive their daughters' educational experiences to inform education service provision. The purpose of this study was to survey parents of school-aged children with Rett syndrome to describe the educational services that they receive and understand parents' perceptions of and satisfaction with the special educational and related services.

Evidence of altered salivary cytokine concentrations in Rett syndrome and associations with clinical severity.

Background: Immune dysregulation may play a role in the development of Rett syndrome (RTT), a neurodevelopmental disorder caused by mutations of the MECP2 gene. Abnormal cytokine concentrations have been documented in the serum of individuals with RTT. Measurement of salivary cytokines has been investigated as a potential alternative approach to measurement in blood and serum, but it is unclear whether salivary cytokine concentrations can provide valid information about systemic immune function in neurodevelopmental disorders.

Preliminary Evidence That Resting State Heart Rate Variability Predicts Reactivity to Tactile Stimuli in Rett Syndrome.

Patients with Rett syndrome may manifest altered pain perception/experience and are vulnerable to conditions associated with chronic pain. Pain response is difficult to measure, however, because of severe communicative impairment. There is also documented autonomic dysfunction, including decreased heart rate variability.