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.

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.

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.

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.

Investigating the Feasibility of a Modified Quantitative Sensory Testing Approach to Profile Sensory Function and Predict Pain Outcomes Following Intrathecal Baclofen Implant Surgery in Cerebral Palsy.

Objectives: Intrathecal baclofen (ITB) pumps used to manage spasticity in children with cerebral palsy (CP) also improve pain outcomes for some but not all patients. The purpose of this clinical feasibility study was to explore whether a quantitative sensory testing approach could a) be modified and used to subgroup individuals into sensory profiles and b) test whether the profiles were related to postimplant pain outcomes (i.e.

Psychometric properties of the brief pain inventory modified for proxy report of pain interference in children with cerebral palsy with and without cognitive impairment.

Introduction: Cerebral palsy (CP) is the most common cause of physical disability in children and is often associated with secondary musculoskeletal pain. Cerebral palsy is a heterogeneous condition with wide variability in motor and cognitive capacities. Although pain scales exist, there remains a need for a validated chronic pain assessment tool with high clinical utility for use across such a heterogeneous patient population with and without cognitive impairment.

A case-controlled comparison of postoperative analgesic dosing between girls with Rett syndrome and girls with and without developmental disability undergoing spinal fusion surgery.

Background: Rett syndrome is associated with severe motor and communicative impairment making optimal postoperative pain management a challenge. There are case reports documenting reduced postoperative analgesic requirement in Rett syndrome.

Aim: The goal of this preliminary investigation was to compare postoperative analgesic management among a sample of girls with Rett syndrome compared to girls with and without developmental disability undergoing spinal fusion surgery.