Investigating the interrater reliability of a novel functional outcome measure for use in the burns intensive care unit: The Functional Assessment for Burns - Critical Care (FAB-CC).

INTRODUCTION: Intensive Care Unit Acquired Weakness challenges the clinical care of critically ill patients. Despite a surge in validated ICU functional outcome measures following the publication of Clinical Guideline 83 'Rehabilitation After Critical Illness' by the National Institute for Health and Care Excellence (2009), there are none composed specifically for use in the Burns ICU. We therefore developed and tested the inter-rater reliability of a burn specific novel functional outcome measure; The Functional Assessment for Burns-Critical Care (FAB-CC). OBJECTIVES: This research aimed to investigate the interrater reliability of the FAB-CC. METHODS: A quantitative reliability study assessed the ability of the FAB-CC to obtain accurate data when utilised by three separate raters (m=3) scoring the same clinical episodes (n=24). RESULTS: The Intraclass Correlation Coefficient (ICC) for the FAB-CC as a complete tool revealed excellent agreement (ICC 0.998; P<0.001, 95% confidence interval 0.996-0.999), with Cronbach's Alpha (α 0.999). Individual components of the FAB-CC displayed excellent agreement (ICC>0.983; P<0.001) with narrow 95% confidence intervals. CONCLUSIONS: The FAB-CC is a novel functional outcome measure that is reliable for use with critically ill burn patients. It has demonstrated real clinical utility in the identification and management of ICU-AW in this unique patient group.

A survey of current management of Benign Paroxysmal Positional Vertigo (BPPV) by physiotherapists' interested in vestibular rehabilitation in the UK.

OBJECTIVES: Benign Paroxysmal Positional Vertigo (BPPV) is the most common cause of dizziness. Extensive research has identified the best assessment and treatment manoeuvres for each subtype of BPPV. Education in vestibular rehabilitation (VR) is inconsistent. It is unclear if the evidence has been adopted by UK physiotherapists in clinical practice and no research has investigated this specifically. DESIGN: An online survey with closed- and open-text answers. PARTICIPANTS: A purposive sample of physiotherapists interested in VR. A response rate of 67% (100/150) was obtained, from which 20 responses were excluded. RESULTS: Participants had good evidence-based awareness in assessment (79/80, 99%) and treatment (72/80, 90%) of posterior BPPV. Horizontal BPPV assessment awareness was lower than treatment (37/80, 46% vs 60/80, 75%). Differential diagnosis was poor in subjective (20/80, 25%) and objective stages of assessment (34/80, 43%). Thirty six percent (29/80) were able to list ≥3 test precautions with all three nystagmus characteristics described by 29% (23/80). Eighty one percent (65/80) encourage activity restrictions post-treatment. Only 28% (22/80) were aware of practice guidelines or Cochrane reviews in BPPV. External courses were rated the top method for learning how to manage BPPV. Lack of peer support (26/77, 34%) was the main challenge faced whilst learning. Recommendations for improving BPPV education included more external courses (23/87, 26%) and competency guidelines (13/87, 15%). CONCLUSIONS: Good awareness of research evidence was observed in some aspects of BPPV management but many areas require development. Translation and implementation of evidence remains poor and suggests changes in education and knowledge dissemination are warranted.

Frequency and circumstances of falls in people with inclusion body myositis: a questionnaire survey to explore falls management and physiotherapy provision.

OBJECTIVES: To survey the incidence and circumstances of falls for people with inclusion body myositis (IBM) in the UK, and to investigate the provision of physiotherapy and falls management. DESIGN: Postal questionnaire survey. SETTING: Participants completed questionnaires at home. PARTICIPANTS: Ninety-four people diagnosed with IBM were screened against the inclusion criteria. Seventy-two potential participants were sent a questionnaire, and 62 were completed and returned. Invited participants were sent an adapted Falls Event Questionnaire pertaining to falls, perceived causes of falls and the provision of physiotherapy. Questionnaires were returned anonymously. MAIN OUTCOME MEASURES: The proportions of respondents who reported a fall or a near fall, along with the frequencies of falls and near falls were calculated. Descriptive data of falls were collected pertaining to location and cause. Data analysis was performed to investigate provision of physiotherapy services. RESULTS: The response rate was 86% [62/72, mean (standard deviation) age 68 (8) years]. Falls were reported by 98% (61/62) of respondents, with 60% (37/62) falling frequently. In this study, age was not found to be an indicator of falls risk or frequency. Twenty-one percent (13/62) of respondents had not seen a physiotherapist in relation to their IBM symptoms, and of those that had, 31% (15/49) had not seen a physiotherapist until more than 12 months after IBM was diagnosed. Only 18% (11/61) of fallers reported that they had received falls management input. CONCLUSIONS: Falls are a common occurrence for people with IBM, independent of age and years since symptoms first presented, and are poorly addressed by appropriate physiotherapy management. National falls guidelines are not being followed, and referral rates to physiotherapy need to improve.

Hand weakness in Charcot-Marie-Tooth disease 1X.

There have been suggestions from previous studies that patients with Charcot-Marie-Tooth disease (CMT) have weaker dominant hand muscles. Since all studies to date have included a heterogeneous group of CMT patients we decided to analyse hand strength in 43 patients with CMT1X. We recorded handedness and the MRC scores for the first dorsal interosseous and abductor pollicis brevis muscles, median and ulnar nerve compound motor action potentials and conduction velocities in dominant and non-dominant hands. Twenty-two CMT1X patients (51%) had a weaker dominant hand; none had a stronger dominant hand. Mean MRC scores were significantly higher for first dorsal interosseous and abductor pollicis brevis in non-dominant hands compared to dominant hands. Median nerve compound motor action potentials were significantly reduced in dominant compared to non-dominant hands. We conclude that the dominant hand is weaker than the non-dominant hand in patients with CMT1X.

Frequency of mutations in the genes associated with hereditary sensory and autonomic neuropathy in a UK cohort.

The hereditary sensory and autonomic neuropathies (HSAN, also known as the hereditary sensory neuropathies) are a clinically and genetically heterogeneous group of disorders, characterised by a progressive sensory neuropathy often complicated by ulcers and amputations, with variable motor and autonomic involvement. To date, mutations in twelve genes have been identified as causing HSAN. To study the frequency of mutations in these genes and the associated phenotypes, we screened 140 index patients in our inherited neuropathy cohort with a clinical diagnosis of HSAN for mutations in the coding regions of SPTLC1, RAB7, WNK1/HSN2, FAM134B, NTRK1 (TRKA) and NGFB. We identified 25 index patients with mutations in six genes associated with HSAN (SPTLC1, RAB7, WNK1/HSN2, FAM134B, NTRK1 and NGFB); 20 of which appear to be pathogenic giving an overall mutation frequency of 14.3%. Mutations in the known genes for HSAN are rare suggesting that further HSAN genes are yet to be identified. The p.Cys133Trp mutation in SPTLC1 is the most common cause of HSAN in the UK population and should be screened first in all patients with sporadic or autosomal dominant HSAN.

De-stabilizing and training effects of foot orthoses in multiple sclerosis.

This study evaluates the effects of dynamic foot orthoses (DFO) on walking and balance performance in people with multiple sclerosis (MS). Sixteen ambulant subjects with MS and ten age-matched healthy control subjects were studied on initial receipt of foot orthoses and after four weeks of daily wear. Walking speed, MS Walking Scale-12 (MSWS-12) and standing balance were assessed with and without orthoses at both these times. During standing, stance width and vision were varied, and performance was quantified using the velocity of the centre of pressure (COP), body sway velocity and the mean COP position relative to the shoe. People with MS walked slower (P <0.001) and showed increased sway when standing (P <0.001). At the first assessment, the foot orthoses caused an increase in sway and a medial and posterior shift of the COP position. At repeat measurement, the DFOs continued to increase sway compared to a shoe only condition. However, MS subjects reported an improvement in the MSWS-12 (P <0.05) and, compared to the initial session, showed decreased sway when eyes were closed both with and without DFOs. Dynamic foot orthoses may increase sway and change COP position by altering foot alignment and/or plantar afferent stimulation. Improvement in body sway over time may be an overall training effect of the DFOs, as MS subjects adapt to the initial de-stabilization.