Abstract
BackgroundSwallowing dysfunction is related to major cause of adverse events and an indicator of shorter survival among patients with neuromuscular disorders (NMD). It is critical to assess the swallowing function during disease progression, however, there are limited tools that can easily evaluate swallowing function without using videofluoroscopic or videoendoscopic examination. Here, we evaluated the longitudinal changes in tongue thickness (TT) and maximum tongue pressure (MTP) among patients with amyotrophic lateral sclerosis (ALS), myotonic dystrophy type 1 (DM1), and Duchenne muscular dystrophy (DMD).MethodsBetween 2010 and 2020, TT and MTP were measured from 21 ALS, 30 DM1, and 14 DMD patients (mean ages of 66.9, 44.5, and 21.4 years, respectively) at intervals of more than half a year. TT was measured, by ultrasonography, as the distance from the mylohyoid muscle raphe to the tongue dorsum, and MTP was determined by measuring the maximum compression on a small balloon when pressing the tongue against the palate. Then we examined the relationship between these evaluations and patient background and swallowing function.ResultsMean follow-up periods were 24.0 months in the ALS group, 47.2 months in the DM1group, and 61.1 months in the DMD group. The DMD group demonstrated larger first TT than the other groups, while the DM1 group had lower first MTP than the ALS group. The ALS group showed a greater average monthly reduction in mean TT than the DM1 group and greater monthly reductions in mean body weight (BW) and MTP than the other groups. Significant differences between the first and last BW, TT, and MTP measures were found only in the ALS group.ConclusionsThis study suggests that ALS is associated with more rapid degeneration of tongue function over several years compared to DMD and DM1.
Highlights
Swallowing dysfunction is related to major cause of adverse events and an indicator of shorter survival among patients with neuromuscular disorders (NMD)
We evaluated the relationship between tongue thickness (TT) and strength in NMD patients at a single time point [4] and found that TT was significantly greater in Duchenne muscular dystrophy (DMD) patients, while maximum tongue pressure (MTP) was lower in myotonic dystrophy type 1 (DM1) patients compared to other NMD groups
Mean functional oral intake scale (FOIS) at the first measurement decreased by the last one, from 6.3 ± 1.3 to 4.6 ± 1.5 in the amyotrophic lateral sclerosis (ALS) group, from 6.1 ± 1.2 to 4.9 ± 1.3 in the Myotonic dystrophy type 1 (DM1) group, from 5.3 ± 1.3 to 3.9 ± 1.8 in the DMD group
Summary
Swallowing dysfunction is related to major cause of adverse events and an indicator of shorter survival among patients with neuromuscular disorders (NMD). Patients with neuromuscular disorders (NMD) often experience problems with swallowing during the course of the illness They require a periodic video fluoroscopic swallowing study (VFSS) or fiberoptic endoscopic evaluation of swallowing (FEES) to reduce the risk of aspiration pneumonia and assess the need for appropriate nutritional support. Tongue hypertrophy which causes chewing and swallowing difficulties needs adequate nutritional support in DMD [1, 2], and tongue muscle deficit is the major factor causing dysphagia in ALS [3]. Monitoring these changes is critical for clinical management during disease progression
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