Health measurement scales

https://archive-ouverte.unige.ch/unige:111231/ATTACHMENT01

ESPEN guideline clinical nutrition in neurology

Bulbar impairment represents a hallmark feature of Amyotrophic Lateral Sclerosis (ALS) that significantly impacts survival and quality of life. Speech and swallowing dysfunction are key contributors to the clinical heterogeneity of ALS and require well-timed and carefully coordinated interventions. The accurate clinical, radiological and electrophysiological assessment of bulbar dysfunction in ALS is one of the most multidisciplinary aspects of ALS care, requiring expert input from speech-language pathologists (SLPs), neurologists, otolaryngologists, augmentative alternative communication (AAC) specialists, dieticians, and electrophysiologists-each with their own evaluation strategies and assessment tools. The need to systematically evaluate the comparative advantages and drawbacks of various bulbar assessment instruments and to develop integrated assessment protocols is increasingly recognized. In this review, we provide a comprehensive appraisal of the most commonly utilized clinical tools for assessing and monitoring bulbar dysfunction in ALS based on the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) evaluation framework. Despite a plethora of assessment tools, considerable geographical differences exist in bulbar assessment practices and individual instruments exhibit considerable limitations. The gaps identified in the literature offer unique opportunities for the optimization of existing and development of new tools both for clinical and research applications. The multicenter validation and standardization of these instruments will be essential for guideline development and best practice recommendations.

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Clinical Measures of Bulbar Dysfunction in ALS

ALS is a relentlessly progressive and fatal disease, with no curative therapies available to date. Symptomatic and palliative care, provided in a multidisciplinary context, still remains the cornerstone of ALS management. However, our understanding of the molecular mechanisms underlying the disease has advanced greatly over the past years, giving new hope for the development of novel diagnostic and therapeutic approaches. Here, we have reviewed the most recent studies that have contributed to improving both clinical management and our understanding of ALS pathogenesis.

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Recent advances in amyotrophic lateral sclerosis

The penetration-aspiration scale (PAS) is an 8-point scale used to characterize the depth and response to airway invasion during videofluoroscopy. Though widely used in the field of deglutition, there is a lack of consensus regarding the statistical properties of the scale. In order to better understand the state of the literature and the statistical use of the PAS, a systematic review was undertaken to descriptively examine trends in statistical and reporting practices of the PAS since its inception. Online databases were searched for studies citing the original PAS article, which yielded 754 unique articles. Of these, 183 studies were included in the review. Results showed inconsistencies in the statistical use of the scale; 79 studies treated the PAS as ordinal, 71 as categorical, and 49 as interval. Ten types of categorizations were identified. Reporting of power analyses (9%), as well as inter- (26%) and intra-rater (17%) reliability, was uncommon. Among studies that administered multiple bolus volumes or consistencies, 55% reported PAS analyses at the participant/group level only. This review confirms the existence of discrepancies in the statistical treatment of the PAS. A lack of consensus among researchers limits comparisons between studies. The approach to handling this scale dictates the statistical tests used, potentially affecting results and interpretations. Consistent application of statistically sound approaches to PAS analyses is vital for the future of deglutition research.

Use of the Penetration-Aspiration Scale in Dysphagia Research: A Systematic Review.

Dysphagia is one of the most critical problems in patients with progressive neuromuscular diseases. However, clinically useful and practical scales to evaluate dysphagia are limited. Therefore, the aim of this study was to develop such a scale. An 8-stage Neuromuscular Disease Swallowing Status Scale (NdSSS) was developed and tested for its inter- and intrarater reliabilities, concurrent validity, and responsiveness. The NdSSS was used to evaluate 134 patients with Duchenne muscular dystrophy (DMD) and 84 patients with amyotrophic lateral sclerosis (ALS). Inter- and intrarater reliabilities were examined with weighted kappa statistics. Concurrent validity was assessed by correlating the NdSSS with the existing scales [Functional Oral Intake Scale (FOIS), Functional Intake LEVEL Scales (FILS), and ALS Functional Rating Scale-Revised Swallow (ALSFRS-R Sw)], using Spearman’s correlation coefficients. Responsiveness was determined with the standardized response mean (SRM). For inter- and intrarater reliabilities, the weighted kappas were 0.95 and 1.00, respectively, for DMD; and 0.98 and 0.98, respectively, for ALS. The NdSSS showed strong correlations with the FOIS (rs = 0.87 for DMD, rs = 0.93 for ALS, p < 0.001), FILS (rs = 0.89 for DMD, rs = 0.92 for ALS, p < 0.001), and ALSFRS-R SW (rs = 0.93, p < 0.001). SRMs were 0.65 for DMD and 1.21 for ALS. The SRM was higher in DMD patients for the NdSSS than for the other scales, while it was similar in ALS patients and the other scales. Our originally developed NdSSS demonstrated sufficient reliability, validity, and responsiveness in patients with DMD and ALS. It is also useful in evaluating dysphagia in patients with progressive neuromuscular diseases.

Development of a new scale for dysphagia in patients with progressive neuromuscular diseases: the Neuromuscular Disease Swallowing Status Scale (NdSSS)

Background: The energy demands in patients with stroke during the subacute phases are unclear. However, this information is essential for appropriate clinical and nutritional management. The aims of this study were to determine the resting energy expenditure (REE) during the subacute phases, examine its relationships with stroke types, location, severity of hemiparesis, and activities of daily living (ADL), and evaluate whether estimation of REE from the Harris-Benedict equation (HB) requires the addition of a ‘stress factor' to capture possible additional REE imposed by stroke. Methods: We measured REE in 95 patients with subacute stroke (53.5 ± 16.6 days post-stroke) with indirect calorimetry, and compared it with predicted values of energy expenditure estimated from the HB (expressed as percentage). Patients were admitted for rehabilitation of their first ischemic or nonsurgical hemorrhagic stroke. The severity of hemiparesis was assessed with the motor items of the Stroke Impairment Assessment Set (SIAS). The ADL was assessed with the Functional Independence Measure (FIM). We compared REE and %HB of the two groups divided by hemiparesis severity and ADL limitation using the Student's t-test, and those of the five groups divided by stroke location. The correlations between REE and the motor items of the SIAS and the FIM score were assessed with the Spearman rank correlation test. A multiple regression analysis for REE was conducted. Results: The average body weight (BW) was 57.1 ± 11.3 kg. The average body mass index (BMI) was 22.5 ± 4.0. The mean REE (%HB) was 1,271 ± 284 kcal/day (106.0 ± 17.3%). REE and %HB of the low ADL group was less than that of the high ADL group (p Conclusions: Analysis of energy expenditure suggests that stroke patients are not hypermetabolic during the subacute phase. The ‘stress factor' in stroke patients during the subacute phase was 1.0-1.1. This finding provides important information for improving nutritional management during the subacute phase in patients with stroke.

Resting Energy Expenditure in Patients with Stroke during the Subacute Phases - Relationships with Stroke Types, Location, Severity of Paresis, and Activities of Daily Living

Objective: Asymmetrical skull deformity is frequently seen in children with cerebral palsy, and may contribute to postural abnormalities and deformities. The aim of this cross-sectional survey was to determine the frequency of asymmetrical skull deformity and its correlation with clinical parameters. Methods: A 10-item checklist for asymmetrical skull deformity, postural abnormalities, and deformities was developed, and its inter-rater reliability was tested. A total of 110 participants aged 1–18 years (mean age 9.3 years (standard deviation 4.7)) was assessed using the checklist. The frequency of asymmetrical skull deformity was analysed and related to the Gross Motor Function Classification System (GMFCS), postural abnormalities, and deformities. Results: The reliability of the checklist was satisfactory (κ > 0.8). Asymmetrical skull deformity was observed in 44 children, 24 showing right and 20 showing left flat occipital deformity. Its frequency was significantly related to GMFCS and with the patterns of asymmetrical posture and deformities (p < 0.05). Children with right flat occipital asymmetrical skull deformity showed predominantly rightward facial direction and right-side-dominant asymmetrical tonic neck reflex, left convex scoliosis, right-side-elevated pelvic obliquity, and left-sided hip dislocation. Those with left flat occipi tal asymmetrical skull deformity demonstrated the reverse tendency. Conclusion: Asymmetrical skull deformity is frequent in cerebral palsy and closely related to asymmetrical posture and deformities. This information will be useful to manage these problems.

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Asymmetric skull deformity in children with cerebral palsy: frequency and correlation with postural abnormalities and deformities.

Purpose Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. Materials and methods The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia. Results Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p Conclusion Patients with BMD have swallowing problems similar to those observed in patients with DMD when matched according to physical functional status. These patients should be evaluated and followed-up for the duration of their disease. Implications for rehabiliation Dysphagia is one of the most critical problems in patients with progressive neuromuscular disease but dysphagia in patients with Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

Ayako Wada

Papers

Development of a new scale for dysphagia in patients with progressive neuromuscular diseases: the Neuromuscular Disease Swallowing Status Scale (NdSSS)

Resting Energy Expenditure in Patients with Stroke during the Subacute Phases - Relationships with Stroke Types, Location, Severity of Paresis, and Activities of Daily Living

Asymmetric skull deformity in children with cerebral palsy: frequency and correlation with postural abnormalities and deformities.

A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

Long-term follow-up of dysphagia in adult patients with Duchenne muscular dystrophy