Characteristics of tongue and pharyngeal pressure in patients with neuromuscular diseases

Figures & data

Table 1 Videofluoroscopic dysphagia scale

Parameter	Coded value		Score
Oral phase score
Lip closure	Intact	0	4
	Inadequate	2
	None	4
Bolus formation	Intact	0	6
	Inadequate	3
	None	6
Mastication	Intact	0	8
	Inadequate	4
	None	8
Apraxia	None	0	4.5
	Mild	1.5
	Moderate	3
	Severe	4.5
Tongue-to-palate contact	Intact	0	10
	Inadequate	5
	None	10
Premature bolus loss	None	0	4.5
	<10%	1.5
	10%–50%	3
	>50%	4.5
Oral transit time	<1.5	0	3
	>1.5	3
Pharyngeal phase score
Triggering of pharyngeal swallow	Normal	0	4.5
	Delayed	4.5
Vallecular residue	None	0	6
	<10%	2
	10%–50%	4
	>50%	6
Laryngeal elevation	Normal	0	9
	Impaired	9
Pyriform sinus residue	None	0	13.5
	<10%	4.5
	10%–50%	9
	>50%	13.5
Coating of pharyngeal wall	No	0	9
	Yes	9
Pharyngeal transit time	<1.0 s	0	6
	>1.0 s	6
Aspiration	None	0	12
	Supraglottic penetration	6
	Subglottic aspiration	12
Total			100

Figure 1 Position of the four sensors of the transducer probe in videomanofluorometry.

Notes: The sensors of channels 1–3 were positioned from the oropharynx to the hypopharynx, while the sensor of channel 4 was positioned at the upper esophageal sphincter.

Figure 2 Maximum pressures in the hypopharynx in channels 1–3 were measured from the highest peak of a waveform to the base line, and pressure change in the UES in channel 4 was measured from a higher peak, that is, maximum closing UES pressure, to the bottom of a waveform, that is, minimum opening UES pressure.

Abbreviation: UES, upper esophageal sphincter.

Figure 3 VF images of typical cases in each group; pyriform sinus residue in a patient with DMD and DM1 (A and B, respectively) and supraglottic penetration in a patient with ALS (C).

Abbreviations: ALS, amyotrophic lateral sclerosis; DM1, myotonic dystrophy type 1; DMD, Duchenne muscular dystrophy; VF, videofluoroscopy.

Table 2 Comparison of the three groups with regard to patient characteristics and swallowing pressures

	DMD group	DM1 group	ALS group	Reference value^Citation11
Age (years)	22.0±5.5	47.8±12.0	68.9±7.1
Men:women	17 men	16:16	10:16
Bulbar onset:limb onset			13:13
Diet-type score (score, number of patients)	2.24 (0, 0; 1, 6; 2, 3; 3, 6; 4, 2)	2.12 (0, 7; 1, 2; 2, 7; 3, 12; 4, 4)	1.92 (0, 3; 1, 10; 2, 2; 3, 8; 4, 3)
Videofluoroscopy score (minimum–maximum)	7.5 (2–16.5)	12.2 (2–27.5)	20.6 (0–59.5)**
Tongue pressure (kPa)	21.7±7.8**	13.9±6.4	13.5±10.2
Data in healthy individuals (age boundaries)^Citation12	41.7±9.7 (20s)	40.4±9.8 (40s)	37.6±8.8 (60s)
Change in hypopharynx pressure (mmHg)				(healthy volunteers)
Channel 1	80.3±64.0	48.2±51.6	71.6±34.0	71.1 (50.2–142.3)
Channel 2	78.3±118.1	28.7±29.6	82.9±43.1	53.5 (20–89.7)
Channel 3	126.7±74.6	76.3±73.9	178.5±79.5	62.5 (47.3–14.8)
Mean of channels 1–3	95.1±42.4	51.1±29.9**	111.0±32.4
Change in UES pressure (mmHg)
Channel 4	217.9±135.4	95.6±92.2**	160.8±75.8	187.4 (70.1–287.2)

Notes:

** p<0.01. Differences in diet-type score and videofluoroscopy score were analyzed using the Steel-Dwass tests. Differences in swallowing pressures were analyzed using the Bonferroni (Dunn) t-tests.

Abbreviations: ALS, amyotrophic lateral sclerosis; DM1, myotonic dystrophy type 1; DMD, Duchenne muscular dystrophy; UES, upper esophageal sphincter.

Table 3 Comparison between the bulbar-onset group and the limb-onset group in the ALS group

	ALS group	Bulbar-onset group	Limb-onset group
Age (years)	68.9±7.1	71.2±6.6	66.5±7.0
Men:women	10:16	5:8	5:8
Disease duration (years)	2.7±3.8	1.4±1.3	3.9±5.1
Body mass index (kg/m^2)	18.4±3.4	19.3±3.8	17.5±2.9
ALSFRS-R (minimum–maximum)	27.8 (13–46)	29.7 (13–46)	25.9 (14–42)
Diet type (score, number of patients)	1.92 (0, 3; 1, 10; 2, 2; 3, 8; 4, 3)	1.54 (0, 1; 1, 7; 2, 2; 3, 3; 4, 0)	2.31 (0, 2; 1, 3; 2, 0; 3, 5; 4, 3)
Videofluoroscopy score (minimum–maximum)	20.6 (0–59.5)	25.0 (0–59.5)	16.2 (2–38.5)
Tongue pressure (kPa)	13.5±10.2	7.46±9.0*	17.6±8.3
Change in hypopharynx pressure (mmHg)
Channel 1	71.6±34.0	61.7±30.2	81.5±35.8
Channel 2	82.9±43.1	92.7±46.9	73.1±38.3
Channel 3	178.5±79.5	155.0±65.0	202.1±88.0
Mean of channels 1–3	111.0±32.4	103.1±34.1	118.9±29.7
Change in UES pressure (mmHg)
Channel 4	160.8±75.8	141.1±78.0	180.5±71.0

Notes:

* p<0.05. Differences in ALSFRS-R, diet-type score and videofluoroscopy score were analyzed using the Steel-Dwass tests. Differences in swallowing pressures were analyzed using the Bonferroni (Dunn) t-tests.

Abbreviations: ALS, amyotrophic lateral sclerosis; ALSFRS-R, ALS functional rating scale; UES, upper esophageal sphincter.

Table 4 Correlations between age and swallowing pressures and among swallowing pressures in the three groups of patients

	DMD group	DM1 group	ALS group
Age vs tongue pressure	R=−0.498, p=0.046*	R=−0.108, p=0.547	R=−0.104, p=0.635
Age vs hypopharynx pressure	R=−0.014, p=0.955	R=−0.019, p=0.914	R=0.044, p=0.826
Age vs UES pressure	R=−0.500, p=0.045*	R=−0.199, p=0.267	R=−0.189, p=0.346
Tongue pressure vs hypopharynx pressure	R=0.105, p=0.688	R=0.421, p=0.016*	R=−0.026, p=0.909
Tongue pressure vs UES pressure	R=0.353, p=0.164	R=0.329, p=0.066	R=0.197, p=0.379
Hypopharynx pressure vs UES pressure	R=−0.277, p=0.282	R=−0.110, p=0.549	R=0.030, p=0.883

Notes:

* p<0.05. The relationships between pressures and age were measured using the Spearman’s rank-order correlation. The relationships between swallowing pressures were measured using the Pearson correlation coefficient.

Abbreviations: ALS, amyotrophic lateral sclerosis; DM1, myotonic dystrophy type 1; DMD, Duchenne muscular dystrophy; UES, upper esophageal sphincter.

Table 5 Correlations between ALSFRS-R and swallowing pressures and among swallowing pressures in the ALS bulbar-onset and limb-onset groups

	ALS group	Bulbar-onset group	Limb-onset group
ALSFRS-R vs tongue pressure	R=0.078, p=0.715	R=−0.025, p=0.943	R=0.084, p=0.767
ALSFRS-R vs hypopharynx pressure	R=0.435, p=0.030*	R=0.437, p=0.130	R=0.593, p=0.040*
ALSFRS-R vs UES pressure	R=−0.034, p=0.864	R=−0.088, p=0.761	R=0.177, p=0.540
BMI vs tongue pressure	R=−0.148, p=0.509	R=−0.087, p=0.823	R=0.095, p=0.758
BMI vs hypopharynx pressure	R=0.294, p=0.144	R=0.535, p=0.060	R=0.162, p=0.596
BMI vs UES pressure	R=−0.049, p=0.811	R=0.252, p=0.407	R=−0.304, p=0.313
Tongue pressure vs hypopharynx pressure	R=−0.026, p=0.909	R=−0.310, p=0.417	R=−0.060, p=0.845
Tongue pressure vs UES pressure	R=0.197, p=0.379	R=0.191, p=0.622	R=−0.137, p=0.656
Hypopharynx pressure vs UES pressure	R=0.030, p=0.883	R=−0.285, p=0.344	R=0.300, p=0.320

Notes:

* p<0.05. The relationships between pressures and ALSFRS-R were measured using the Spearman’s rank-order correlation. The relationships between swallowing pressures were measured using the Pearson correlation coefficient.

Abbreviations: ALS, amyotrophic lateral sclerosis; ALSFRS-R, ALS functional rating scale; BMI, body mass index; UES, upper esophageal sphincter.

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