Progressive supranuclear palsy: Advances in diagnosis and management

Progressive supranuclear palsy (PSP) is a complex clinicopathologic disease with no current cure or disease modulating therapies that can only be definitively confirmed at autopsy. Growing understanding of the phenotypic diversity of PSP has led to expanded clinical criteria and new insights into etiopathogenesis that coupled with improved in vivo biomarkers makes increased access to current clinical trials possible. Current standard-of-care treatment of PSP is multidisciplinary, supportive and symptomatic, and several trials of potentially disease modulating agents have already been completed with disappointing results. Current ongoing clinical trials target the abnormal aggregation of tau through a variety of mechanisms including immunotherapy and gene therapy offer a more direct method of treatment. Here we review PSP clinicopathologic correlations, in vivo biomarkers including MRI, PET, and CSF biomarkers. We additionally review current pharmacologic and non-pharmacologic methods of treatment, prior and ongoing clinical trials in PSP. Newly expanded clinical criteria and improved specific biomarkers will aid in identifying patients with PSP earlier and more accurately and expand access to these potentially beneficial clinical trials.     

Effectiveness of robotic exoskeletons for improving gait in children with cerebral palsy: A systematic review

BackgroundRobotic exoskeletons have been developed to assist locomotion and address gait abnormalities in children with cerebral palsy (CP). These wearable assistive devices provide powered assistance to the lower-extremity joints, as well as support and stability.Research QuestionDoes exoskeleton-assisted walking improve gait in children with CP?MethodsThe PRISMA guidelines were used to conduct this systematic review. Articles were obtained in a search of the following electronic databases: Embase, CINAHL Complete, PubMed, Web of Science and MEDLINE. Studies investigating spatiotemporal, kinematic, kinetic, muscle activity and/or physiological parameters during exoskeleton-assisted walking in children with CP were included. All articles were assessed for methodological quality using an adapted version of the Quality Assessment Tool for Before-After (Pre-Post) Studies with No Control Group, provided by the National Institutes of Health (NIH).ResultsThirteen studies were included. They involved the use of the following exoskeletons: tethered knee exoskeleton, pediatric knee exoskeleton (P.REX), untethered ankle exoskeleton, WAKE-Up ankle module, WAKE-Up ankle & knee module and unilateral ankle exosuit. Methodological quality varied, with key limitations in sample size and allocated time to adapt to the exoskeleton. There was a consensus that robotic exoskeletons improve gait given careful optimisation of exoskeleton torque and sufficient exoskeleton practice time for each participant. Improvements in gait included reduced metabolic cost of walking, increased walking speed, and increased knee and hip extension during stance. Furthermore, exoskeletons with an actuated ankle module were shown to promote normal ankle rocker function.SignificanceRobotic exoskeletons have the potential to improve the mobility of CP children and may therefore increase community participation and improve quality of life. Future work should involve larger controlled intervention studies utilising robotic exoskeletons to improve gait in children with CP. These studies should ensure sufficient exoskeleton practice time for each participant.     

Adjunct therapies to improve outcomes after botulinum toxin injection in children: A systematic review

BackgroundBotulinum toxin (BTX) injection alone is not sufficient to treat spasticity in children, notably those with cerebral palsy; thus, there is an emerging trend for adjunct therapies to offer greater outcomes than BTX alone.ObjectiveThe aim of this systematic review was to evaluate the general effectiveness of adjunct therapies regardless of their nature in children with spasticity.MethodsMedline, Cochrane and Embase databases were searched from January 1980 to March 15, 2018 for reports of parallel-group trials (randomized controlled trials [RCTs] and non-RCTs) assessing adjunct therapies after BTX injection for treating spasticity in children. Two independent reviewers extracted data and assessed the risk of bias by using the PEDro scale for RCTs and Downs and Black scale (D&B) for non-RCTs.ResultsOverall, 20 articles involving 662 participants met the inclusion criteria. The average quality was good for the 16 RCTs (mean PEDro score 7.4 [SD 1.6]) and poor to moderate for the 4 non-RCTs (D&B score 9 to 17). Adjunct therapies consisted of casting/posture, electrical stimulation, resistance training and rehabilitation programmes. Casting associated with BTX injection improved the range of passive and active motion and reduced spasticity better than did BTX alone (9 studies), with a follow-up of 1 year. Resistance training enhanced the quality and performance of muscles without increasing spasticity. Only 3 rehabilitation programmes were studied, with encouraging results for activities.ConclusionLower-limb posture with casting in children has a high level of evidence, but the long-term efficacy of short-leg casting needs to be evaluated. A comparison between the different modalities of casting is missing, and studies specifically devoted to testing the different kinds of casting are needed. Moreover, the delay to casting after BTX injection is not clear. Data on electrical stimulation are not conclusive. Despite the small number of studies, resistance training could be an interesting adjunct therapy notably to avoid loss of strength after BTX injection. Rehabilitation programmes after BTX injection still need to be evaluated.     

‘It’s important that we learn too’: Empowering parents to facilitate participation in physical activity for children and youth with disabilities

Aim: The actions and behaviors of parents have been identified as key factors that influence a child’s participation in physical activity. However, there is limited knowledge of how parents can be supported to embody facilitative roles. This study aimed to explore how an ecological intervention encourages parents of children with disabilities to develop as facilitators, to enable ongoing physical activity participation in a child’s local environment.

Methods: A qualitative design using grounded theory was employed. Forty four parents (26 mothers, 18 fathers) of 31 children with a range of disabilities (mean age 12y 6m (SD 2y 2m); 18 males) partaking in the Local Environment Model intervention at Beitostolen Healthsports Centre in Norway participated in the study. Data were derived from the triangulation of semi-structured interviews and participant observation. Data analysis was an iterative approach of constant comparison, where data collection, memo writing, open, axial and selective coding analysis, were undertaken simultaneously. Findings were consolidated into a model describing the central phenomenon and its relationship to other categories.

Results: Thematic concepts uncovered in this study describe a social process of parent learning and empowerment, comprising three primary components; (i) active ingredients of the intervention that enabled learning and empowerment to transpire, (ii) parent learning and empowerment as a process, and (iii) related outcomes.

Conclusion: A family-centered approach, encompassing family-to-family support, may enhance physical activity participation outcomes for children and youth with disabilities.     

Fractures of the humeral shaft

Fractures of the humeral shaft are relatively common and can occur in any a variety of age groups, and due to a variety of mechanisms of injury. The anatomy of the humeral shaft is vital to understand, to be able to plan management of these fractures: the intricate relationship of the radial nerve with the humeral shaft in particular, plays an important role in these fractures. Though non-operative measures are commonly employed for humeral shaft fractures routinely, there are some indications for operative management. There remain a variety of operative interventions available to a surgeon, all with pros and cons associated. This review aims to look in detail at the anatomy of the humeral shaft, the types and sites of fractures, the evidence and surgical methodology of the most common surgical interventions, including a discussion about the surgical complications, particularly a radial nerve palsy.     

Problèmes posés par la verticalité de l’enfant paralysé cérébral et rééducation selon l’approche Bobath

During normal development a baby progressively learns to function against gravity eventually resulting in a postural control in an upright position. This was described by Doctor K. Bobath as the normal postural reflex mechanism. Due to the presence of diverse motor disorders in children with cerebral palsy this process is modified. Here we will explain the different disorders in relation to their classification and describe their treatment using the Bobath Approach.