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P.10.13 Swallowing evaluation in adult neuromuscular patients
792
Abstracts / Neuromuscular Disorders 23 (2013) 738–852
Three distinct analyses were performed considering the 3 domains (D1 Standing position and transfers, D2 Axial and proximal and D3 Distal motor function) of the MFM using RUMM 2030 software with a partial credit model. The original MFM failed to meet the model expectations in each domain. The reduction to a three-categorie response (0,1,2) scale in 20 items with disordered thresholds, produced ordered response thresholds for 18 items. Seven misfitting items were identified as not fitting and removed. Finally, the Rasch-scaled 10-item MFM D1, 9-item MFM D2 and 6-item MFM D3 conformed adequately to the Rasch measurement model. The Rasch-scaled-MFM D1 was well targeted to the patient population with quite similar mean location for items (0.000) and person (0.316) whereas the Rasch-scaled-MFM D2 and D3 were better targeted for more severely affected patients, as expected from the domains covered. Reliability coefficients of the Rasch scaled MFM suggest sufficient ability for the total score D1 (0.9) and D2 (0.8) and D3 (0.7) to distinguish between groups for research use. The results provide evidence for the measurement properties of the Rasch-scaled MFMCDM promoting its use as outcome measures in clinical trials for patients with congenital disorders of muscle. A raw score-to-Rasch person measure conversion is supplied for investigators. http://dx.doi:10.1016/j.nmd.2013.06.540
P.10.11 Validity of the 2 min walk test as an outcome measure in individuals with CMD and other neuromuscular diseases M. Jain 1, R. Logaraj 1, M. Waite 1, C.Y. Shieh 2, J. Dastgir 3, S. Donkervoort 3, M. Leach 3, C. Bonnemann 3 1 National Institutes of Health, Clinical Research Center, Rehabiliation Medicine Department, Bethesda, United States; 2 National Institutes of Health, Bethesda, United States; 3 National Institutes of Health, National Institute of Neurological Disorders and Stroke, Bethesda, United States
P.10.12 Difference of the mechanism of dysphagia between Duchenne muscular dystrophy and myotonic dystrophy type 1 H. Furuya 1, G. Umemoto 2, T. Kikuta 2, H. Arahata 3, N. Fujii 3 1 Neuro-Muscular Center, NHO Omuta Hospital, Fukuoka, Japan; 2 Department of Oral and Maxillofacial Surgery, Faculty of Medicine, Fukuoka University, Fukuoka, Japan; 3 Department of Neurology, Neuro-Muscular Center, National Omuta Hospital, Fukuoka, Japan Duchenne muscular dystrophy (DMD) and myotonic dystrophy type 1 (DM1) show clinically similar myopathic symptoms on initial examination, but usually very different swallowing difficulties become apparent. In this study we aimed to demonstrate the distinctive features of dysphagia in patients with DM1 and DMD. Diet-type score (DTS) and maximum tongue pressure (MTP) were measured in 20 DM1 and 24 DMD patients; all patients were also examined by video-fluoroscopy (VF). We used VF to measure the range of hyoid bone excursion (RHBE) during pharyngeal transit time and the area of pharyngeal residue (APR) after the first swallow. RHBE and APR values for DM1 patients were significantly greater than those for DMD patients. DTS and MTP did not differ between patients. A significant correlation was observed between DTS, MTP and RHBE in DMD patients, but not in DM1 patients. Our findings reveal the differences in the distinctive features of dysphagia in DM1 and DMD. Adjustments in the diet of DMD patients in accordance with swallowing ability could be useful. http://dx.doi:10.1016/j.nmd.2013.06.542
P.10.13 Swallowing evaluation in adult neuromuscular patients S. Serel, N. Demir, A.A. Karaduman Hacettepe University, Physiotherapy and Rehabilitation, Ankara, Turkey
The congenital muscular dystrophies (CMD) are a heterogeneous group of early onset degenerative diseases of muscle leading to significant and progressive weakness and eventually affecting mobility and respiratory function. There is a need for an effective outcome measure to monitor patients’ clinical progression and capture the effect of interventional clinical trials on patients’ strength and endurance. Currently, the 6-min walk test is used for this purpose, but there are limitations of this test for the CMD patient population, including the patients’ inability to complete the test because of significant weakness, contractures, and fatigue. We propose that shortening the test to 2 min would alleviate these issues without compromising reliability. In this study, we administered the 6 min walk test to 55 patients with various neuromuscular disorders including CMD, who ranged in age from 6 to 61 years, between 2010 and 2012. We compared the distance traveled in the initial 2 min with that traveled during the entire 6 min and found them to be highly correlated (Pearson’s r = .964, p < .05). The distance walked in 2 min was shown to be highly predictive of the distance walked in 6 min, demonstrating that patients maintained a consistent pace throughout the test. The distances traveled in 2 and 6 min also inversely correlated with other functional measures, including timed floor to stand (r for 2 min = .560, p < .05; r for 6 min = .595, p < .05), the timed 10 meter run (r for 2 min = .765, p < .05; r for 6 min = .795, p < .05), and timed 4 stair ascent (r for 2 min = .662, p < .05; r for 6 min = .686, p < .05) and descent (r for 2 min = .544, p < .05; r for 6 min = .566, p < .05). This study inversely correlates the 2 min and 6 min walk times with functional tasks and also provides data to support the shortening of the 6 min walk test to 2 min in patients with neuromuscular disorders including CMD.
The aim was to investigate the swallowing physiology in adult neuromuscular patients who have swallowing disorders. Videofluoroscopic swallowing evaluation (VFSE) was done in adult neuromuscular patients who admitted to our clinic with swallowing disorders. Tongue elevation, retraction, swallowing reflex, hyolaryngeal elevation, the presence of residue, penetration, aspiration and esophageal motility disorders were evaluated. Tongue elevation, retraction and hyolaryngeal elevation was scored as absent, inadequate and exact. Swallowing reflex was noted as normal, mild, moderate and severe delay. Residue, penetration, aspiration and esophageal motility disorders were noted as absent and exact. The diet was determined as orally and non-orally according to swallowing evaluation. 31 patients with the mean age of 58.3 ± 12.7 years, mean height of 164.26 ± 9.6 cm and mean weight of 60.88 ± 15.5 kg were evaluated. 51.6% of patients were Amyotrophic Lateral Sclerosis, 22.6% of patients were motor neuron patients, 16.1% of patients were Myasthenia Gravis and 9.7% of patients were myopathic patients. 54.8% of patients had inadequate tongue elevation, 61.3% had inadequate tongue retraction and 51.6% had inadequate hyolaryngeal elevation. Despite 71% had normal swallowing reflex, 9.7% had mild, 9.7% had moderate and 9.7% had severe delay in swallowing reflex. 71% of patients had residue, 51.6% had penetration and 29% had aspiration. Esophageal motility disorders were determined in 35.5% of patients. 71% of patients (n: 22) were recommended oral intake and 29% (n: 9) were recommended non-oral feeding according to swallowing evaluation. Our results show that the swallowing disorders are based on inadequate peripheral structures in these patients. Although swallowing reflexes were normal, problems occurred. Deterioration in swallowing functions should be considered because of the progression of diseases.
http://dx.doi:10.1016/j.nmd.2013.06.541
http://dx.doi:10.1016/j.nmd.2013.06.543
Abstracts / Neuromuscular Disorders 23 (2013) 738–852
Three distinct analyses were performed considering the 3 domains (D1 Standing position and transfers, D2 Axial and proximal and D3 Distal motor function) of the MFM using RUMM 2030 software with a partial credit model. The original MFM failed to meet the model expectations in each domain. The reduction to a three-categorie response (0,1,2) scale in 20 items with disordered thresholds, produced ordered response thresholds for 18 items. Seven misfitting items were identified as not fitting and removed. Finally, the Rasch-scaled 10-item MFM D1, 9-item MFM D2 and 6-item MFM D3 conformed adequately to the Rasch measurement model. The Rasch-scaled-MFM D1 was well targeted to the patient population with quite similar mean location for items (0.000) and person (0.316) whereas the Rasch-scaled-MFM D2 and D3 were better targeted for more severely affected patients, as expected from the domains covered. Reliability coefficients of the Rasch scaled MFM suggest sufficient ability for the total score D1 (0.9) and D2 (0.8) and D3 (0.7) to distinguish between groups for research use. The results provide evidence for the measurement properties of the Rasch-scaled MFMCDM promoting its use as outcome measures in clinical trials for patients with congenital disorders of muscle. A raw score-to-Rasch person measure conversion is supplied for investigators. http://dx.doi:10.1016/j.nmd.2013.06.540
P.10.11 Validity of the 2 min walk test as an outcome measure in individuals with CMD and other neuromuscular diseases M. Jain 1, R. Logaraj 1, M. Waite 1, C.Y. Shieh 2, J. Dastgir 3, S. Donkervoort 3, M. Leach 3, C. Bonnemann 3 1 National Institutes of Health, Clinical Research Center, Rehabiliation Medicine Department, Bethesda, United States; 2 National Institutes of Health, Bethesda, United States; 3 National Institutes of Health, National Institute of Neurological Disorders and Stroke, Bethesda, United States
P.10.12 Difference of the mechanism of dysphagia between Duchenne muscular dystrophy and myotonic dystrophy type 1 H. Furuya 1, G. Umemoto 2, T. Kikuta 2, H. Arahata 3, N. Fujii 3 1 Neuro-Muscular Center, NHO Omuta Hospital, Fukuoka, Japan; 2 Department of Oral and Maxillofacial Surgery, Faculty of Medicine, Fukuoka University, Fukuoka, Japan; 3 Department of Neurology, Neuro-Muscular Center, National Omuta Hospital, Fukuoka, Japan Duchenne muscular dystrophy (DMD) and myotonic dystrophy type 1 (DM1) show clinically similar myopathic symptoms on initial examination, but usually very different swallowing difficulties become apparent. In this study we aimed to demonstrate the distinctive features of dysphagia in patients with DM1 and DMD. Diet-type score (DTS) and maximum tongue pressure (MTP) were measured in 20 DM1 and 24 DMD patients; all patients were also examined by video-fluoroscopy (VF). We used VF to measure the range of hyoid bone excursion (RHBE) during pharyngeal transit time and the area of pharyngeal residue (APR) after the first swallow. RHBE and APR values for DM1 patients were significantly greater than those for DMD patients. DTS and MTP did not differ between patients. A significant correlation was observed between DTS, MTP and RHBE in DMD patients, but not in DM1 patients. Our findings reveal the differences in the distinctive features of dysphagia in DM1 and DMD. Adjustments in the diet of DMD patients in accordance with swallowing ability could be useful. http://dx.doi:10.1016/j.nmd.2013.06.542
P.10.13 Swallowing evaluation in adult neuromuscular patients S. Serel, N. Demir, A.A. Karaduman Hacettepe University, Physiotherapy and Rehabilitation, Ankara, Turkey
The congenital muscular dystrophies (CMD) are a heterogeneous group of early onset degenerative diseases of muscle leading to significant and progressive weakness and eventually affecting mobility and respiratory function. There is a need for an effective outcome measure to monitor patients’ clinical progression and capture the effect of interventional clinical trials on patients’ strength and endurance. Currently, the 6-min walk test is used for this purpose, but there are limitations of this test for the CMD patient population, including the patients’ inability to complete the test because of significant weakness, contractures, and fatigue. We propose that shortening the test to 2 min would alleviate these issues without compromising reliability. In this study, we administered the 6 min walk test to 55 patients with various neuromuscular disorders including CMD, who ranged in age from 6 to 61 years, between 2010 and 2012. We compared the distance traveled in the initial 2 min with that traveled during the entire 6 min and found them to be highly correlated (Pearson’s r = .964, p < .05). The distance walked in 2 min was shown to be highly predictive of the distance walked in 6 min, demonstrating that patients maintained a consistent pace throughout the test. The distances traveled in 2 and 6 min also inversely correlated with other functional measures, including timed floor to stand (r for 2 min = .560, p < .05; r for 6 min = .595, p < .05), the timed 10 meter run (r for 2 min = .765, p < .05; r for 6 min = .795, p < .05), and timed 4 stair ascent (r for 2 min = .662, p < .05; r for 6 min = .686, p < .05) and descent (r for 2 min = .544, p < .05; r for 6 min = .566, p < .05). This study inversely correlates the 2 min and 6 min walk times with functional tasks and also provides data to support the shortening of the 6 min walk test to 2 min in patients with neuromuscular disorders including CMD.
The aim was to investigate the swallowing physiology in adult neuromuscular patients who have swallowing disorders. Videofluoroscopic swallowing evaluation (VFSE) was done in adult neuromuscular patients who admitted to our clinic with swallowing disorders. Tongue elevation, retraction, swallowing reflex, hyolaryngeal elevation, the presence of residue, penetration, aspiration and esophageal motility disorders were evaluated. Tongue elevation, retraction and hyolaryngeal elevation was scored as absent, inadequate and exact. Swallowing reflex was noted as normal, mild, moderate and severe delay. Residue, penetration, aspiration and esophageal motility disorders were noted as absent and exact. The diet was determined as orally and non-orally according to swallowing evaluation. 31 patients with the mean age of 58.3 ± 12.7 years, mean height of 164.26 ± 9.6 cm and mean weight of 60.88 ± 15.5 kg were evaluated. 51.6% of patients were Amyotrophic Lateral Sclerosis, 22.6% of patients were motor neuron patients, 16.1% of patients were Myasthenia Gravis and 9.7% of patients were myopathic patients. 54.8% of patients had inadequate tongue elevation, 61.3% had inadequate tongue retraction and 51.6% had inadequate hyolaryngeal elevation. Despite 71% had normal swallowing reflex, 9.7% had mild, 9.7% had moderate and 9.7% had severe delay in swallowing reflex. 71% of patients had residue, 51.6% had penetration and 29% had aspiration. Esophageal motility disorders were determined in 35.5% of patients. 71% of patients (n: 22) were recommended oral intake and 29% (n: 9) were recommended non-oral feeding according to swallowing evaluation. Our results show that the swallowing disorders are based on inadequate peripheral structures in these patients. Although swallowing reflexes were normal, problems occurred. Deterioration in swallowing functions should be considered because of the progression of diseases.
http://dx.doi:10.1016/j.nmd.2013.06.541
http://dx.doi:10.1016/j.nmd.2013.06.543