Children with developmental central hypotonia have reduced muscle tone secondary to non-progressive damage to the brain or brainstem. Children may have transient delays, mild or global functional impairments, and the lack of a clear understanding of this diagnosis makes evaluating appropriate interventions challenging.

This overview aimed to systematically describe the best available evidence for tools to identify and evaluate children with developmental central hypotonia aged 2 months to 6 years.

A systematic review of systematic reviews or syntheses was conducted with electronic searches in PubMed, Medline, CINAHL, Scopus, Cochrane Database of Systematic Reviews, Google Scholar, and PEDro and supplemented with hand-searching. Methodological quality and risk-of-bias were evaluated, and included reviews and tools were compared and contrasted. Three systematic reviews, an evidence-based clinical assessment algorithm, three measurement protocols, and two additional measurement tools were identified.

For children aged 2 months to 2 years, the Hammersmith Infant Neurological Examination has the strongest measurement properties and contains a subset of items that may be useful for quantifying the severity of hypotonia.

For children aged 2-6 years, a clinical algorithm and individual tools provide guidance. Further research is required to develop and validate all evaluative tools for children with developmental central hypotonia.

Gross Motor Family Report: Refinement and evaluation of psychometric properties

Elton D. D. Magalhães, Peter Rosenbaum, Marilyn Wright, F. Virginia Wright, Lesley Pritchard, Kennea M. A. Ayupe, Ana Carolina de Campos, Rosane S. Morais, Hercules R. Leite, Paula S. C. Chagas

Abstract

Aim
To refine the Gross Motor Family Report (GM-FR) using parents' input and to evaluate its psychometric properties.

Method
In this measurement study, 12 parents of children and adolescents with cerebral palsy (CP), aged 2 to 18 years, classified in all levels of the Gross Motor Function Classification System (GMFCS), were interviewed about their experience completing the GM-FR (content validity). Parents' feedback was used to refine the measure which was then completed by 146 families to evaluate internal consistency, and discriminative and concurrent validity. Forty-six parents completed the GM-FR again, 7 to 30 days later, to evaluate test–retest reliability.

Results
GM-FR scoring, pictures, descriptions, and the total number of items were revised based on parents' feedback. The GM-FR version 2.0 demonstrated high internal consistency (Cronbach's α = 0.99), no floor/ceiling effects, and excellent test–retest reliability (intraclass correlation coefficient = 0.99). GM-FR scores discriminated between GMFCS levels (p less than 0.05) and were strongly negatively correlated with GMFCS level (r = −0.92; p less than 0.001). GM-FR scores correlated positively and strongly with the Gross Motor Function Measure-66 (r = 0.94; p less than 0.001) and the Pediatric Evaluation of Disability Inventory – Computer Adaptive Test mobility domain (r = 0.93; p less than 0.001).

Interpretation
Active participation of families in the GM-FR's development facilitated creation of a family-friendly instrument. This study provides strong evidence of reliability and validity to support GM-FR use in clinical practice and research for assessing gross motor performance of children and adolescents with CP.

https://onlinelibrary.wiley.com/doi/10.1111/dmcn.16042

Professor Gareth Baynam is a globally recognised clinical geneticist, researcher, and advocate for rare diseases. He is the Director of the Rare Care Centre at Perth Children's Hospital and the Head of the Western Australian Register of Developmental Anomalies.

With a career dedicated to improving the diagnosis, management, and care of individuals with rare and genetic conditions, he has been at the forefront of integrating cutting-edge technologies such as genomics, artificial intelligence, and precision medicine into healthcare.

Professor Baynam is also a leader in Indigenous health initiatives, championing equitable access to rare disease diagnostics and treatment. Through his work with organizations such as the Global Commission to End the Diagnostic Odyssey for Children with a Rare Disease and the European Rare Diseases Research Alliance (ERDERA), he continues to drive global collaborations and innovations that aim to transform rare disease care.

Anita R Gross, Kenneth A Olson, Jan Pool, Annalie Basson, Derek Clewley, Jenifer L Dice, Nikki Milne

• PMID: 38855972
• PMCID: PMC11216248
• DOI: 10.1080/10669817.2024.2332026

Abstract

Introduction: An international taskforce of clinician-scientists was formed by specialty groups of World Physiotherapy - International Federation of Orthopaedic Manipulative Physical Therapists (IFOMPT) & International Organisation of Physiotherapists in Paediatrics (IOPTP) - to develop evidence-based practice position statements directing physiotherapists clinical reasoning for the safe and effective use of spinal manipulation and mobilisation for paediatric populations (<18 years) with varied musculoskeletal or non-musculoskeletal conditions.

Method: A three-stage guideline process using validated methodology was completed: 1. Literature review stage (one scoping review, two reviews exploring psychometric properties); 2. Delphi stage (one 3-Round expert Delphi survey); and 3. Refinement stage (evidence-to-decision summative analysis, position statement development, evidence gap map analyses, and multilayer review processes).

Results: Evidence-based practice position statements were developed to guide the appropriate use of spinal manipulation and mobilisation for paediatric populations. All were predicated on clinicians using biopsychosocial clinical reasoning to determine when the intervention is appropriate.1. It is not recommended to perform:• Spinal manipulation and mobilisation on infants.• Cervical and lumbar spine manipulation on children.•Spinal manipulation and mobilisation on infants, children, and adolescents for non-musculoskeletal paediatric conditions including asthma, attention deficit hyperactivity disorder, autism spectrum disorder, breastfeeding difficulties, cerebral palsy, infantile colic, nocturnal enuresis, and otitis media.2. It may be appropriate to treat musculoskeletal conditions including spinal mobility impairments associated with neck-back pain and neck pain with headache utilising:• Spinal mobilisation and manipulation on adolescents;• Spinal mobilisation on children; or• Thoracic manipulation on children for neck-back pain only.3. No high certainty evidence to recommend these interventions was available.Reports of mild to severe harms exist; however, risk rates could not be determined.

Conclusion: Specific directives to guide physiotherapists' clinical reasoning on the appropriate use of spinal manipulation or mobilisation were identified. Future research should focus on trials for priority conditions (neck-back pain) in children and adolescents, psychometric properties of key outcome measures, knowledge translation, and harms.

Early Detection and Early Intervention - where are we now (and what does the future hold)?

The last time we had Alicia on the pod, we spoke about the Cochrane Review she led titled “Early developmental intervention programmes provided post hospital discharge to prevent motor and cognitive impairment in preterm infants” which was published in 2024. In this week’s episode, we thought we’d ask Alicia about the state of early intervention right now and what the provision of therapy looks like within our current context of early detection and early intervention for children with cerebral palsy.

There have been some rather significant developments in the early detection and early intervention space over the past 20 years. From the rapid technological advances to the value of co-design and involvement of people with lived experience, we now have some impressive evidence to guide our clinical pathways. However, what is very clear now is the vital importance of implementation. The industry has generated substantial knowledge that now needs to be implemented into practice with one particularly important aspect that we must include - family involvement and well-being.

Alicia speaks ever so passionately about our role as therapists and I cannot help but to feel even more compelled to ensure that the family is at the centre of everything we do. It is exciting to know that we have the evidence now, so it’s time to put it into practice and it can start in your very next session.

https://findanexpert.unimelb.edu.au/profile/27041-alicia-spittle

Implementing Clinical Practice Guidelines for Improving Function in Cerebral Palsy: Development of a Fidelity Tool

In this study, the research team developed a 21-item fidelity tool to help clinicians implement evidence-based guidelines for improving function in children with cerebral palsy. Designed for goal setting, intervention, and reflection, the tool was found to be practical and useful but faced challenges like time constraints and family expectations. The study highlights the need for education, self-reflection, and organisational support to bridge the gap between research and practice.

Abstract
Purpose: To develop a fidelity tool to support the implementation of clinical practice guidelines to improve physical function for children and young people with cerebral palsy.

Methods: Fidelity tool development followed a 5-step process: the pilot study, using a mixed-methods action research approach, and including focus groups, questionnaires, and field notes.

Results: A 21-item fidelity tool representing the core components of the clinical practice guidelines was developed, including subsections of goal setting, intervention, and elements seen throughout therapeutic intervention. Clinicians and supervisors reported this tool as acceptable and feasible, especially when used as a self-reflection tool.

Conclusions: A fidelity tool has been developed that clinicians, supervisors and organizations can use to reflect on current practice and plan for changes to align practice with guidelines to improve function in children with cerebral palsy.

https://pubmed.ncbi.nlm.nih.gov/39378353/

Professor Nick Gottardo walks us through medulloblastomas - a comprehensive master class for clinicians and researchers. Expect to learn about early signs and symptoms, diagnosis, treatment and prognosis. Dr Nick breaks this down beautifully.

What was really clear throughout our discussion was that collaboration is key. Given the incidence of medulloblastomas and the specific subtypes that fall within this category, it would not be possible to develop effective treatments that minimise detrimental side effects without coming together with centres across the world.

This episode is truly special so if you want to know the current state of the evidence when it comes to treating medulloblastomas - this is a must listen episode.

All about knowledge translation

A special episode with our illustrious hosts before we catch up with Professor Nick Gottardo, Professor Alicia Spittle, Professor Andrew Whitehouse, Dr Gareth Baynam, Assoc. Professor Nikki Milne and Assoc. Clinical Professor Anita Gross amongst many others!