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

Abstract

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.

Trends in Research Literature Describing Dysphagia in Motor Neuron Diseases (MND): A Scoping Review

“…Observations from radiographic imaging and endoscopy include delayed swallow onset [15,39,41–44], the occurrence of penetration/aspiration [13,15,16,18,27,33,39,41–62] and post-swallow residue [15,16,33,39,41,42,44,47,49,52,53,57–63]. Additional reports from VFSS indicate reduced hyolaryngeal movement [15,33,43,45,53,64] and incomplete laryngeal closure [45,39]. Still, the majority of studies reporting swallowing physiology/function present observations in descriptive terms, limiting our ability to compare results across studies or measure discrete degrees of change.…”

Screening and evaluation tools of dysphagia in children with neuromuscular diseases: a systematic review

“…From the remaining 39 studies, 35 were excluded after full‐text analysis. A final four were included in this review …”

“…In turn, the correlation between the NdSSS and Penetration‐Aspiration Scale was not significant in patients with DMD. For NdSSS, there is no specific indication or result in paediatric patients …”

Cross-Cultural Validation of the Italian Version of the Functional Oral Intake Scale

“…Firstly, it is acknowledged that there are some shortcomings with the FOIS itself. The scale, originally validated on a stroke population and widely used in other dysphagia populations [14-17, 21], refers to the individual’s oral intake status but does not incorporate information on safety of swallowing at these levels. As a descriptive scale, therefore, it was difficult to validate against another similar reference scale, as none exists.…”

“…The FOIS has been administered in different populations, such as in patients with amyotrophic lateral sclerosis [14], in pediatric populations [15] as well as in patients with head and neck cancer [16]. In addition, the FOIS has been used as outcome measure in swallowing treatment studies in Parkinson’s disease [17, 18].…”