PRECISION ALS-an integrated pan European patient data platform for ALS.

Amyotrophic Lateral Sclerosis (ALS) is an incurable neurodegenerative condition. Despite significant advances in pre-clinical models that enhance understanding of disease pathobiology, translation of candidate drugs to effective human therapies has been disappointing. There is increasing recognition of the need for a precision medicine approach toward drug development, as many failures in translation can be attributed in part to disease heterogeneity in humans. PRECISION-ALS is an academic industry collaboration between clinicians, Computer Scientists, Information engineers, technologists, data scientists and industry partners that will address the key clinical, computational, data science and technology associated research questions to generate a sustainable precision medicine based approach toward new drug development. Using extant and prospectively collected population based clinical data across nine European sites, PRECISION-ALS provides a General Data Protection Regulation (GDPR) compliant framework that seamlessly collects, processes and analyses research-quality multimodal and multi-sourced clinical, patient and caregiver journey, digitally acquired data through remote monitoring, imaging, neuro-electric-signaling, genomic and biomarker datasets using machine learning and artificial intelligence. PRECISION-ALS represents a first-in-kind modular transferable pan-European ICT framework for ALS that can be easily adapted to other regions that face similar precision medicine related challenges in multimodal data collection and analysis.

Musculoskeletal pain patterns and association between dizziness symptoms and pain in patients with long term dizziness - a cross-sectional study.

BACKGROUND: The impact of long-term dizziness is considerable both on the personal level and in society and may lead to self-imposed restrictions in daily activities and social relations due to fear of triggering the symptoms. Musculoskeletal complaints seem to be common in persons with dizziness, but studies addressing these complaints as a widespread occurrence, are scarce. This study aimed to examine the occurrence of widespread pain in patients with long-term dizziness and investigate the associations between pain and dizziness symptoms. Further, to explore whether diagnostic belonging is related to the occurrence of pain. METHODS: This cross-sectional study was conducted in an otorhinolaryngology clinic and included 150 patients with persistent dizziness. The patients were categorized into three groups: episodic vestibular syndromes, chronic vestibular syndromes, and non-vestibular group. The patients completed questionnaires on dizziness symptoms, catastrophic thinking, and musculoskeletal pain when entering the study. Descriptive statistics were used to describe the population, and associations between pain and dizziness were investigated by linear regression. RESULTS: Pain was reported by 94.5% of the patients. A significantly higher prevalence of pain was reported in all the ten pain sites examined compared to the general population. Number of pain sites and pain intensity were associated with the dizziness severity. Number of pain sites was also associated with dizziness-related handicap, but not with catastrophic thinking. There was no association between pain intensity and dizziness-related handicap or catastrophic thinking. Pain was equally distributed in the diagnostic groups. CONCLUSION: Patients with long-term dizziness have a considerably higher prevalence of pain and number of pain sites than the general population. Pain co-exists with dizziness and is associated with dizziness severity. These findings may indicate that pain should be systematically assessed and treated in patients with persisting dizziness.

Hearing threshold and physical performance in older people: a cross-sectional study from the HUNT4 cohort.

PURPOSE: To evaluate the association between increased hearing loss and reduced physical performance in older people. METHODS: Cross-sectional population-based study using data from the fourth wave of the Trøndelag Health Survey (HUNT4) in Norway. Data were obtained from the subproject HUNT4 Hearing which collected audiometric data of people > 70 years (N = 13,197). Analyses were performed on all participants who had completed audiometry and measured balance using the Short Physical Performance Battery (SPPB), which was scored from 0 (worst score) to 12. The hearing threshold was expressed as a pure tone average (PTA). Associations between the hearing threshold for the best and worst ear and physical performance were analyzed by linear regression models adjusted for age, sex, education, diabetes, and cardiovascular disease. Hearing threshold was indicated with steps of 10 dB. RESULTS: Of 13,197 eligible participants, 4101 who completed audiometry and SPPB (52.3% women. mean age 76.3 years) were included. The analyses revealed an association between reduced SPPB and increased hearing threshold in the best ear (b = - 0.296; 95% CI - 0.343 to - 0. 249; P < 0.001) and the worst ear (b = - 0.229; 95% CI - 0.270 to - 0.189; P < 0.001). CONCLUSIONS: In this population study, we found that the increased hearing threshold was associated to reduced physical performance as measured by SPPB. The association seemed to be strongest for the best ear. The association between hearing threshold and physical performance illustrates the importance of assessing physical performance in people with hearing loss to prevent the risk of falls and disability. The underlying causes of the associations between hearing loss and poorer physical performance are not fully understood and should be further investigated. LEVEL OF EVIDENCE: Level 3.

Pilates-based exercises for gait and balance in ambulant children with cerebral palsy: feasibility and clinical outcomes of a randomised controlled trial.

PURPOSE: To determine if Pilates-based exercise classes could be feasible and effective in changing gait kinematics and balance in ambulant children with Cerebral Palsy (CP). MATERIALS AND METHODS: A single-blind multi-centre randomised controlled trial compared a four-week, twice-weekly Pilates-based exercise class to a usual exercise control, for ambulant children with CP. Clinical outcome measures were three-dimensional trunk and lower limb kinematics during walking on level ground, uneven ground and crossing an obstacle; and clinical balance measures. Feasibility outcomes were adherence and enjoyment. RESULTS: Forty-six children (29 male, mean age 10 years 8 months (range 7-17 years), 23 per group) participated. After the four-week intervention, there were no significant between-group differences in trunk or lower limb gait kinematics. Differences were detected in Berg Balance Scale (1.38 points, 95% CI 0.58-2.18) and Functional Walking Test (1.40 points, 95% CI 0.58-2.22), but they were less than the minimum clinically important difference and therefore clinically insignificant. Median class attendance was 5/8 classes. CONCLUSION: Pilates-based exercises did not change lower limb or trunk kinematics during walking in children with CP and had a clinically insignificant impact on balance. Lower than anticipated adherence prompts consideration of more flexible delivery of future interventions. Implications for RehabilitationAmbulant children with CP can experience impairment of trunk control, negatively impacting balance and gait.In this study, Pilates-based exercise classes did not change kinematics of the trunk or lower limbs during walking and led to negligible improvement in functional balance.Children did not manage to do their Home Exercise Programme, indicating that Pilates-based exercise should be delivered within supervised practice.Children missed on average one in three classes due to unforeseen circumstances, so this should be anticipated when planning group classes.

Toward a Digital Health Intervention for Vestibular Rehabilitation: Usability and Subjective Outcomes of a Novel Platform.

Digital technologies are increasingly available and are reducing in cost. There is an opportunity to move to a digital health approach in vestibular rehabilitation (VR), but there is a paucity of suitable systems available and a consequent lack of evidence to support their use. This study aimed to investigate a novel digital platform developed specifically for VR (comprising clinician software, a wearable sensor, and a patient-facing app). Participants (n = 14, 9F:5M, mean age 59) with vestibular dysfunction and imbalance used the app for treatment, and therapists (n = 4) used the platform to deliver VR in the form of individualized exercise programmes over a mean of 17.4 ± 8.8 weeks. Outcomes included the system usability scale, the patient enablement instrument (PEI), change in subjective symptoms (numerical rating scales), percentage adherence to prescribed exercise, and a semi-structured interview on utility. A significant reduction was found in symptoms of vertigo/dizziness (p < 0.004), imbalance (p < 0.002), oscillopsia (p < 0.04), and anxiety (p < 0.02) after use. System usability scores were high for both clinicians (mean 85/100) and participants (mean 82.7/100) and high enablement was reported (mean PEI 6.5/12). Overall percentage adherence to the exercise prescription was highly variable and ranged from 4 to 78% when measured digitally. At semi-structured interviews, participants reported a high level of acceptance and satisfaction with digital delivery, and no adverse events were recorded. When COVID-19 restrictions eased, 2 participants trialed the head sensor with the application and found it highly usable. Further research is required to investigate the efficacy and how the wearable sensor impacts the delivery of care.

Prevalence and distribution of musculoskeletal pain in patients with dizziness-A systematic review.

BACKGROUND AND PURPOSE: Musculoskeletal disorders are among the leading causes of disability globally, but their role in patients with dizziness and imbalance is not well understood or explored. Such knowledge may be important as musculoskeletal pain and dizziness can mutually influence each other, leading to a complex condition requiring more comprehensive approaches to promote successful recovery. We conducted a systematic review to examine the extent and characteristic of reported musculoskeletal pain in patients with dizziness. METHODS: A comprehensive literature search in Medline, Embase, Cochrane, Scopus, Amed, Google Scholar, SveMed+, and Web of Science was conducted in March 2021. Inclusion criteria were studies examining patients with a vestibular diagnosis, patients with cervicogenic dizziness and patients included based on having dizziness as a symptom; and reported musculoskeletal pain. Data regarding age, sex, sample size, diagnosis and musculoskeletal pain was extracted. The Crowe Critical Appraisal Tool was used for assessing methodical quality of the included studies. RESULTS: Out of 1507 screened studies, 16 studies met the inclusion criteria. The total sample consisted of 1144 individuals with dizziness. The frequency of patients reporting pain ranged between 43% and 100% in the included studies. Pain intensity were scored between 5 and 7 on a 0-10 scale. Pain in the neck and shoulder girdle was most often reported, but musculoskeletal pain in other parts of the body was also evident. DISCUSSION: In the included studies, musculoskeletal pain was highly prevalent in patients with dizziness, with pain intensity that may have a moderate to severe interference with daily functioning. Pain in the neck and shoulder is well documented, but there are few studies addressing musculoskeletal pain in additional parts of the body. More research is needed to understand the relations between dizziness and musculoskeletal pain.

The patient's perspective of remote respiratory assessments during the COVID-19 pandemic.

Forced vital capacity (FVC) is an essential respiratory measurement for assessment and monitoring of patients with Amyotrophic Lateral Sclerosis (ALS). Our clinic rapidly implemented remote assessment of FVC after COVID-19 related restrictions on respiratory testing were imposed, using mini-spirometers and video consultation. We sought to evaluate the patient's experiences of performing remote respiratory assessments to guide future development and optimisation of the service. Twenty-five patients completed surveys. The mean age was 65.2 years and average time from diagnosis was 17.04 (2-99) months. Seventy-two percent (n = 18) required help from a caregiver to perform the tests. Ninety-two percent (n = 23) of patients reported that overall, they were satisfied and were happy to continue with remote respiratory assessment. Reducing the number of clinic visits for review and assessment was valued by 92% (n = 23) and reducing the risk associated with COVID-19 was valued by 96% (n = 24). The highest frequency reported as acceptable for performing the remote breathing assessments was monthly (60%, n = 15), followed by every second month (28%, n = 7). Remote respiratory testing is viewed positively by patients. These technologies used in combination with video-consultations and other novel forms of remote monitoring implemented in response to the COVID-19 crisis will continue to be valuable tools for clinical care in future. However, further evaluation of the validity of remote respiratory assessment is required.

Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Updated Clinical Practice Guideline From the Academy of Neurologic Physical Therapy of the American Physical Therapy Association.

BACKGROUND: Uncompensated vestibular hypofunction can result in symptoms of dizziness, imbalance, and/or oscillopsia, gaze and gait instability, and impaired navigation and spatial orientation; thus, may negatively impact an individual's quality of life, ability to perform activities of daily living, drive, and work. It is estimated that one-third of adults in the United States have vestibular dysfunction and the incidence increases with age. There is strong evidence supporting vestibular physical therapy for reducing symptoms, improving gaze and postural stability, and improving function in individuals with vestibular hypofunction. The purpose of this revised clinical practice guideline is to improve quality of care and outcomes for individuals with acute, subacute, and chronic unilateral and bilateral vestibular hypofunction by providing evidence-based recommendations regarding appropriate exercises. METHODS: These guidelines are a revision of the 2016 guidelines and involved a systematic review of the literature published since 2015 through June 2020 across 6 databases. Article types included meta-analyses, systematic reviews, randomized controlled trials, cohort studies, case-control series, and case series for human subjects, published in English. Sixty-seven articles were identified as relevant to this clinical practice guideline and critically appraised for level of evidence. RESULTS: Based on strong evidence, clinicians should offer vestibular rehabilitation to adults with unilateral and bilateral vestibular hypofunction who present with impairments, activity limitations, and participation restrictions related to the vestibular deficit. Based on strong evidence and a preponderance of harm over benefit, clinicians should not include voluntary saccadic or smooth-pursuit eye movements in isolation (ie, without head movement) to promote gaze stability. Based on moderate to strong evidence, clinicians may offer specific exercise techniques to target identified activity limitations and participation restrictions, including virtual reality or augmented sensory feedback. Based on strong evidence and in consideration of patient preference, clinicians should offer supervised vestibular rehabilitation. Based on moderate to weak evidence, clinicians may prescribe weekly clinic visits plus a home exercise program of gaze stabilization exercises consisting of a minimum of: (1) 3 times per day for a total of at least 12 minutes daily for individuals with acute/subacute unilateral vestibular hypofunction; (2) 3 to 5 times per day for a total of at least 20 minutes daily for 4 to 6 weeks for individuals with chronic unilateral vestibular hypofunction; (3) 3 to 5 times per day for a total of 20 to 40 minutes daily for approximately 5 to 7 weeks for individuals with bilateral vestibular hypofunction. Based on moderate evidence, clinicians may prescribe static and dynamic balance exercises for a minimum of 20 minutes daily for at least 4 to 6 weeks for individuals with chronic unilateral vestibular hypofunction and, based on expert opinion, for a minimum of 6 to 9 weeks for individuals with bilateral vestibular hypofunction. Based on moderate evidence, clinicians may use achievement of primary goals, resolution of symptoms, normalized balance and vestibular function, or plateau in progress as reasons for stopping therapy. Based on moderate to strong evidence, clinicians may evaluate factors, including time from onset of symptoms, comorbidities, cognitive function, and use of medication that could modify rehabilitation outcomes. DISCUSSION: Recent evidence supports the original recommendations from the 2016 guidelines. There is strong evidence that vestibular physical therapy provides a clear and substantial benefit to individuals with unilateral and bilateral vestibular hypofunction. LIMITATIONS: The focus of the guideline was on peripheral vestibular hypofunction; thus, the recommendations of the guideline may not apply to individuals with central vestibular disorders. One criterion for study inclusion was that vestibular hypofunction was determined based on objective vestibular function tests. This guideline may not apply to individuals who report symptoms of dizziness, imbalance, and/or oscillopsia without a diagnosis of vestibular hypofunction. DISCLAIMER: These recommendations are intended as a guide to optimize rehabilitation outcomes for individuals undergoing vestibular physical therapy. The contents of this guideline were developed with support from the American Physical Therapy Association and the Academy of Neurologic Physical Therapy using a rigorous review process. The authors declared no conflict of interest and maintained editorial independence.Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1, available at: http://links.lww.com/JNPT/A369).

Correlations between measures of ALS respiratory function: is there an alternative to FVC?

Background: An ongoing longitudinal study in six European sites includes a 3-monthly assessment of forced vital capacity (FVC), slow vital capacity (SVC), peak cough flow (PCF), and Sniff nasal inspiratory pressure (SNIP). The aim of this interim analysis was to assess the potential for SNIP to be a surrogate for aerosol generating procedures given COVID-19 related restrictions. Methods: This was a prospective observational study. Patients attending six study sites with King's Stage 2 or 3 ALS completed baseline FVC/SVC/SNIP/PCF and repeated assessments 3 monthly. Data were collected from March 2018 to March 2020, after which a COVID-19 related study suspension was imposed. Correlations between the measures were calculated. A Bayesian multiple outcomes random-effects model was constructed to investigate rates of decline across measures. Results: In total, 270 cases and 828 assessments were included (Mean age 65.2 ± 15.4 years; 32.6% Female; 60% Kings stage 2; 81.1% spinal onset). FVC and SVC were the most closely correlated outcomes (0.95). SNIP showed the least correlation with other metrics 0.53 (FVC), 0.54 (SVC), 0.60 (PCF). All four measures significantly declined over time. SNIP in the bulbar onset group showed the fastest rate of decline. Discussion: SNIP was not well correlated with FVC and SVC, probably because it examines a different aspect of respiratory function. Respiratory measures declined over time, but differentially according to the site of onset. SNIP is not a surrogate for FVC and SVC, but is a complementary measure, declining linearly and differentiating spinal and bulbar onset patients.

Vestibular rehabilitation in Europe: a survey of clinical and research practice.

Vestibular rehabilitation (VR) is practiced across Europe but little in this area has been quantified. The aim of this study was to investigate current VR assessment, treatment, education, and research practices. This was an online, cross-sectional survey with 39 VR specific questions and four sections: demographics, current practice, education, and research. The survey was disseminated through the Dizzynet network to individual therapists through country-specific VR special interest groups. Results were analysed descriptively. A thematic approach was taken to analyse open questions. A total of 471 individuals (median age 41, range 23 - 68 years, 73.4% women), predominately physiotherapists (89.4%) from 20 European countries responded to the survey. They had worked for a median of 4 years (range < 1 - 35) in VR. The majority (58.7%) worked in hospital in-patient or out-patient settings and 21.4% in dedicated VR services. Most respondents specialized in neurology, care of the elderly (geriatrics), or otorhinolaryngology. VR was reported as hard/very hard to access by 48%, with the main barriers to access identified as lack of knowledge of health care professionals (particularly family physicians), lack of trained therapists, and lack of local services. Most respondents reported to know and treat benign paroxysmal positional vertigo (BPPV 87.5%), unilateral vestibular hypofunction (75.6%), and cervicogenic dizziness (63%). The use of vestibular assessment equipment varied widely. Over 70% used high-density foam and objective gait speed testing. Over 50% used dynamic visual acuity equipment. Infrared systems, Frenzel lenses, and dynamic posturography were not commonly employed (< 20%). The most frequently used physical outcome measures were the Clinical Test of the Sensory Interaction of Balance, Functional Gait Assessment/Dynamic Gait Index, and Romberg/Tandem Romberg. The Dizziness Handicap Inventory, Visual Analogue Scale, Falls Efficacy Scale, and the Vertigo Symptom Scale were the most commonly used patient reported outcome measures. Adaptation, balance, and habituation exercises were most frequently used (> 80%), with virtual reality used by 15.6%. Over 70% reported knowledge/use of Semont, Epley and Barbeque-Roll manoeuvres for the treatment of BPPV. Most education regarding VR was obtained at post-registration level (89.5%) with only 19% reporting pre-registration education. There was strong (78%) agreement that therapists should have professionally accredited postgraduate certification in VR, with blended learning the most popular mode. Three major research questions were identified for priority: management of specific conditions, effectiveness of VR, and mechanisms/factors influencing vestibular compensation and VR. In summary, the survey quantified current clinical practice in VR across Europe. Knowledge and treatment of common vestibular diseases was high, but use of published subjective and objective outcome measures as well as vestibular assessment varied widely. The results stress the need of improving both training of therapists and standards of care. A European approach, taking advantage of best practices in some countries, seems a reasonable approach.

Congenital muscular torticollis: the reliability of visual estimation in the assessment of cervical spine active rotation and head tilt by physiotherapists and the impact of clinical experience.

There is a lack of reliable and valid measurement tools to assess neck function in infants with congenital muscular torticollis, and most physiotherapists use visual estimation, which has not been adequately tested for reliability in this population. We examined the reliability of visual estimation of head tilt and active neck rotation in the upright position, on infants with congenital muscular torticollis by physiotherapists. We recruited 31 infants and 26 physiotherapists. Therapists rated videos of infants' head position in the frontal plane (tilt) and transverse plane (active rotation) using visual estimation, on two occasions at least one week apart. Overall, inter-rater reliability was good (mean ICC, 0.68 ± 0.20; mean SEM, 5.1° ± 2.1°). Rotation videos had better reliability (mean ICC, 0.79 ± 0.14) than head tilt videos (mean ICC, 0.58 ± 0.20). Intra-rater reliability was excellent (mean ICC, 0.85 ± 0.08). Both head tilt and rotation had excellent reliability (mean ICC, 0.84 ± 0.08 for head tilt and 0.85 ± 0.09 for rotation). There was no correlation between intra-rater reliability and clinical experience.Conclusion Visual estimation had excellent intra-rater reliability in the assessment of neck active rotation and head tilt on infants with congenital muscular torticollis. Visual estimation had acceptable inter-rater reliability in the assessment of neck active rotation but not of head tilt. There was a wide variation in reliability with no correlation between reliability and clinical experience. Assessment tools for head tilt that are more psychometrically robust should be developed. What is Known: • A thorough assessment of infants presenting with torticollis is essential, using assessment tools with robust psychometric properties • Visual estimation is the most commonly used method of assessment of neck function in infants with torticollis What is New: • Visual estimation had excellent intra-rater reliability in the assessment of neck active rotation and head tilt in the upright position in videos of infants and acceptable inter-rater reliability in the assessment of rotation but not of head tilt • Physiotherapists' clinical experience had minimal relationship with reliability.

Psychometric properties and domains of postural control tests for individuals with knee osteoarthritis: a systematic review.

Individuals with knee osteoarthritis (OA) commonly demonstrate impaired postural control. We aimed to systematically review psychometric properties of tests that assess postural control in people with knee OA and secondly, to determine domains of postural control measured by these tests. PubMed, CINAHL, Embase and Web of Science databases were searched to January 2019. Two independent review authors screened references according to eligibility criteria. The Consensus-based Standards for the selection of health Measurement Instruments were used to evaluate study methodological quality. Postural control tests identified from included studies were assessed to determine postural control constructs using the Systems Framework for Postural Control identifying nine domains. Following the screening of 2643 references, five cross-sectional studies and one longitudinal study (n = 408) were included. Four studies evaluated reliability, validity or responsiveness of three clinical measures: Community Balance and Mobility Scale, Star Excursion Balance Test and Tinetti Performance Oriented Mobility Assessment. Two assessed reliability or validity of a force platform. Results showed that evidence for reliability, validity and responsiveness of all these postural measures is limited. The most common domain of postural control assessed was 'static stability'. No study used measures that evaluated all nine postural control domains. Our results highlight limited investigation of psychometric properties specific to postural control in knee OA. The few existing ones have limitations regarding psychometric robustness and completeness regarding different domains of postural control. Future research should focus on verifying the quality of existing tools to evaluate postural control in knee OA for research and clinical purposes.

Balance, gait and dizziness in adult cochlear implant users: A cross sectional study.

Objective: Cochlear implantation is associated with vestibular impairment due to the close proximity of the structures. The aim of the study was to quantify dizziness/vertigo, gaze instability, balance and gait problems in a sample of adult cochlear implant (CI) users.Methods: An observational, cross sectional study evaluated subjective dizziness (Vestibular Rehabilitation Benefit Questionnaire (VRBQ)), balance confidence (Activities Specific Balance Questionnaire (ABC)), gait (Functional Gait Assessment (FGA) and 10m walk test), balance (Equitest Sensory Organisation Test (SOT)), and computerised dynamic visual acuity (DVA). The Dix Hallpike test was performed to test for benign paroxysmal positional vertigo (BPPV).Results: Twenty participants (n=10F), 2.8(±2.7) years post implantation, with mean age 59.3(±15.8) years were assessed. Subjective dizziness (VRBQ) was low (15.0% (±15.5)) and balance confidence was high (ABC: 82.1%±14.9). FGA scores (25.1 ± 4.4) and gait speed (1.8 (±0.3) m/sec) were below normal. Dx Hallpike was positive in 3. Gaze instability was found in 50% (DVA loss, 0.29 (± 0.16) LogMAR), while 79% demonstrated balance impairment (mean SOT score, 57.8%±14.5), with 42% falling on SOT condition 5.Discussion: Evidence of vestibular dysfunction was identified in these adult CI users.Conclusion: Access to vestibular function assessment and rehabilitation is required in adult CI users.

Gaze stabilisation exercises in vestibular rehabilitation: review of the evidence and recent clinical advances.

Gaze stabilisation exercises are a mainstay of vestibular rehabilitation (VR). They are frequently prescribed to patients with vestibular hypofunction with the aim of improving the gain of the vestibular ocular reflex, improving visual acuity during head movement, and reducing the symptoms of dizziness and vertigo. This review will examine current evidence that has evaluated the efficacy of gaze stabilisation exercises on VOR gain, dynamic visual acuity and oscillopsia. In addition, recent experimental studies that have employed digital technologies and newer forms of gaze stabilisation exercises will be reviewed. The implications for future clinical practice and research in the area of VR will be discussed.

Longitudinal analysis of sniff nasal inspiratory pressure assessed using occluded and un-occluded measurement techniques in amyotrophic lateral sclerosis and primary lateral sclerosis.

Objective: Sniff nasal inspiratory pressure (SNIP) is a commonly used clinical measure of respiratory impairment in amyotrophic lateral sclerosis (ALS), which is used to guide the initiation of noninvasive ventilation (NIV). SNIP can be completed with either an occluded or an un-occluded contralateral nostril. The aim of this study was to compare occluded and un-occluded SNIP measurements and to examine the decline in occluded SNIP over time compared to the ALSFRS-R respiratory subscore. Methods: This was a prospective longitudinal study examining occluded and un-occluded SNIP scores in ALS and PLS patients recorded between 2001 and 2018. Bland and Altman graphs were plotted for occluded vs. un-occluded SNIP measurements taking account of the repeated measures nature of the data. Longitudinal models were constructed as linear mixed effects multi-level models with follow-up in ALS limited to 6 years. Results: SNIP measured with an occluded contralateral nostril was systematically higher than with an un-occluded nostril. SNIP measured using both methods declined non-linearly, particularly after 2-3 years. The best fit model for decline in occluded SNIP included a main effect and interaction between site of onset and time, with age and diagnostic delay as independent variables. This showed a linear decline in spinal onset with a floor effect in bulbar-onset ALS. Conclusion: SNIP measured with an occluded and un-occluded contralateral nostril is not interchangeable, which is relevant in interpreting thresholds for initiation of NIV. SNIP declines non-linearly, which is explained in spinal onset ALS by age and diagnostic delay, but an apparent floor effect remains in bulbar onset.

A randomised controlled trial of multimodal physiotherapy versus advice for recent onset, painful cervical radiculopathy - the PACeR trial protocol.

BACKGROUND: A research gap exists for optimal management of cervical radiculopathy in the first 12 weeks and short term natural history of the condition is somewhat unclear, although thought to be favourable. The primary aim of this assessor blinded, superiority, 2 parallel group randomised controlled trial is to investigate the effects of a 4 week physiotherapy programme (6-8 sessions) of manual therapy, exercise and upper limb neural unloading tape, compared to a control of weekly phone advice; on disability, pain and selected biopsychosocial measures, in acute and sub-acute cervical radiculopathy patients. A secondary aim is to identify whether any baseline variables, symptom duration or group allocation can predict outcome. METHODS: Participants are recruited from GP referrals in an urban setting, from a neurosurgery non-urgent waiting list and from self-referral through Facebook advertising. Eligible participants (n = 64) are diagnosed with radiculopathy based on a clinical prediction rule and must have symptoms of unilateral, single level, radiculopathy for between 2 and 12 weeks, without having yet received physiotherapy. Random 1:1 group allocation (using variable block sizes), allocation concealment, blinded assessment and intention to treat analysis are being employed. Treatment is provided by clinical specialist physiotherapists in primary and secondary care settings. Outcomes are measured at baseline, 4 (primary endpoint) and 12 weeks. Participants' report of pain, disability and their rating of recovery is also recorded by telephone interview at 6 months. Statistical analysis of between group differences will be performed with ANOVAs and MANOVAs, and multivariable regression analysis will be undertaken to explore predictor variables. Ethical approval for this study has been received from the Beaumont Hospital and Irish College of General Practitioners Research Ethics Committees. The trial is registered at ClinicalTrials.gov (NCT02449200). DISCUSSION: An internal pilot study to test retention and recruitment strategies led to trial expansion and this is now a multi centre trial involving 5 clinical sites. TRIAL REGISTRATION: NCT02449200 . Registered 20/05/15.

Measurement properties of instruments for assessment of cervical spine function in infants with torticollis: a systematic review.

The aim of this study was to systematically review the measurement properties of instruments which assess cervical spine function in infants with torticollis. Electronic searches were performed in MEDLINE, CINAHL, Embase, Web of Science and the Cochrane Library, combining three constructs ('torticollis', 'cervical spine assessment' and 'measurement properties'). Two reviewers independently rated the methodological quality and the quality of measurement properties of identified articles, using both the COSMIN (COnsensus-based Standards for the selection of health status Measurement INstruments) checklist and quality criteria for measurement properties. Five studies, using six instruments, met the inclusion criteria and were analysed. Included instruments were the goniometer, electronic pendular goniometer, protractor, still photography, Muscle Function Scale and a range of motion limitation scale. All studies assessed reliability, and one study also assessed content validity and hypothesis testing. The methodological quality of the studies varied from poor to excellent according to the COSMIN checklist. Two instruments were found to have good measurement properties from high-quality studies: still photography for the assessment of habitual head tilt in supine and the Muscle Function Scale for the assessment of side-flexor muscle function in lateral head righting.Conclusion: This systematic review identified two reliable tools for the assessment of cervical spine function in infants with torticollis. Further research is required to assess the measurement properties of tools already described in the literature and to develop further tools for use in this population. What is known? • A thorough assessment of the infant presenting with torticollis is essential, in order to correctly diagnose, rule out 'red flags' and manage appropriately • Assessment tools need to have robust measurement properties in order to be of value for clinical practice and research What is new? • This systematic review identified two valid and reliable tools for the assessment of cervical spine function in infants with torticollis • Further research is required to assess the measurement properties of tools already described in the literature and to develop further tools for use in infants with torticollis.

Kinematic differences between children with obstetric brachial plexus palsy and healthy controls while performing activities of daily living.

BACKGROUND: Residual shoulder dysfunction and deformity impacts on functional performance in children with obstetric brachial plexus palsy. Clinical understanding of upper limb dynamic movement patterns is difficult with observation alone. This case-control study describes the significant kinematic differences between children with obstetric brachial plexus palsy compared to typically developing children while performing tasks of the modified Mallet Scale. METHODS: Eleven children with obstetric brachial plexus palsy (mean 10 years, range 7-15 years, Narakas group I-III) and 10 typically developing children (mean 9 years 9 months, range 6-15 years) completed three-dimensional upper limb motion analysis using the acromion method to track dynamic scapular movement. Kinematic data were captured by a 4-CODA cx1 optoelectronic tracking system. Participants performed three trials of the modified Mallet scale tasks. Local coordinate systems, segment and joint rotations were defined as recommended by the International Society of Biomechanics. FINDINGS: Joint rotation angles against time of the glenohumeral, thoracohumeral and scapulohumeral joints were calculated. Kinematic findings demonstrated increased internal rotation in all postures, reduced glenohumeral excursion, habitual "trumpet" posture of glenohumeral abduction/elevation and variability in movement strategies. Scapulohumeral rhythm during abduction task was 1.88:1 in typically developing children and 1.04:1 in children with obstetric brachial plexus palsy. INTERPRETATION: Children with obstetric brachial plexus palsy demonstrate deficient external rotation in all tasks. Despite increased postural internal rotation, ability to move through internal rotation range is compromised. The glenohumeral joint showed the greatest range deficit, contributing to abnormal scapulohumeral rhythm. Future sub-group analysis of Narakas Classification is recommended.

Reliability of 3D upper limb motion analysis in children with obstetric brachial plexus palsy.

Kinematics, measured by 3D upper limb motion analysis (3D-ULMA), can potentially increase understanding of movement patterns by quantifying individual joint contributions. Reliability in children with obstetric brachial plexus palsy (OBPP) has not been established. OBJECTIVE: This study aimed to determine between session reliability and measurement errors of 3D-ULMA using the acromion method (AM) in children with OBPP. APPROACH: Ten participants (mean 10 years, range 7-15 years, Narakas classification I-III) completed 3D-ULMA on two occasions, mean interval of 8.6 d (±2.8 d). Kinematic data were captured by a 4-CODA cx1 optoelectronic tracking system. Participants performed three trials of the modified Mallet scale tasks. Local coordinate systems, segment and joint rotations were defined as recommended by the International Society of Biomechanics. The intraclass correlation coefficient (ICC 2,K) and standard error of measurement (SEM) were calculated for task duration, range and joint angle at point of task achievement (PTA). MAIN RESULTS: Results indicated poor reliability for spatiotemporal parameters and range. Moderate to excellent reliability at PTA was observed in 19/60 variables (ICC: 0.77-0.98; SEM: 3.5°-10.4°). The Abduction Task had the highest (ICC: 0.79-0.98; SEM: 3.5°-10.3°) with External Rotation the lowest reliability. Glenohumeral and thoracohumeral elevation had the most consistent reliability. Scapular protraction/retraction had consistently poor reliability (ICC: 0-0.72; SEM: 3.5°-10.2°) with axial rotation also poor (ICC: 0.00-0.91; SEM: 6.3°-32.8°). This study determined inconsistent test-retest reliability of 3D-ULMA, using AM, to track dynamic performance of functional tasks in children with OBPP. It is the first study to outline measurement error in this population. This information permits more reliable interpretation of future studies of kinematic patterns in children with OBPP.