- Email: [email protected]
T.P.45
Abstracts / Neuromuscular Disorders 24 (2014) 791–924 Concurrent validity for the MFM32, HFMS, and NSAA was strong as evidenced by large correlations (ranging 0.76–0.93, p < 0.0001) between the three measures. The MFM32 and HFMS scores were able to discriminate individuals by ambulatory status (p < 0.001). The HFMS, NSAA, and MFM32 had a floor effect, but the MFM32 floor effect was limited to Domain 1 (standing and transfers). The MFM32 also had a ceiling effect distributed across all 3 domains. All three measures correlated highly, but the HFMS and MFM32 may be redundant for use in the same study. The NSAA is useful as a complementary outcome measure in ambulatory individuals. http://dx.doi:10.1016/j.nmd.2014.06.373
T.P.43 Effects of proximal and distal muscle weakness on performance in neuromuscular diseases I. Alemdaroglu 1, P. Kaya 2, O. Yilmaz 2, A.A. Karaduman 2, H. Topaloglu 2 1 Bezmy Alem University, Istanbul, Turkey; 2 Hacettepe University, Ankara, Turkey The aim of this study was to investigate and compare the performance exposure due to different muscle weakness involvement in proximally and distally affected neuromuscular diseases (NMD’s). A total of 40 pediatric patients with different NMD’s who were grouped based on proximal (Group 1) and distal (Group 2) muscle weakness involvement were included in the study. The children between Grade 1 and 3 according to Brooke Lower Extremity Functional Classification (BLEFC) were taken under assessments. Lower extremity regional and total muscular strengths were assessed with manual muscle testing. 10 m walking time (s) and 6 Minute Walk Test (6MWT) (m) were used to evaluate performance level. The relationship between muscle weakness and performance levels was analyzed. 20 patients with proximal (Duchenne Muscular Dystrophy-DMD) and 20 with distal muscle weakness (Hereditary Sensory Motor Neuropathy) who were respectively 9 ± 3.1 and 12.9 ± 3.3 years old were included in the study. Lower extremity total muscle strengths were statistically different with 81.5 ± 18.7 in group 1 and 98.7 ± 16.5 in group 2 (z = 2.764; p < 0.05). There were not any differences in 10 m walking time (z = 0.122) and 6MWT distances (z = 0.609) between two groups (p > 0.05). There were positive correlations between lower extremity muscles strength scores except for ankle girdle and 6MWT distance (p < 0.01); negative, moderate correlations between 10 m walking time and regional/total lower extremity strength scores in group 1 (p < 0.01). There was not any relationship between regional/total muscular strengths and performance tests in Group 2 (p > 0.05). Muscle weakness distribution is an important performance determinative in NMD’s. Performance is more effected in proximal muscle weakness involvement than distal at early stages of the disease. http://dx.doi:10.1016/j.nmd.2014.06.374
T.P.44 Influence of a two-year steroid treatment on body composition as measured by Dual X-ray Absorptiometry in boys with Duchenne Muscular Dystrophy C. Vuillerot 1, P. Braillon 2, S. Fontaine-Carbonnel 2, P. Rippert 3, E. Andre 4, J. Iwaz 5, I. Poirot 2, C. Berard 2 1 Hospices Civils de Lyon, Universite´ de Lyon, CNRS UMR 5558, Bron, France; 2 Hospices Civils de Lyon, Hospital Femme Mere Enfant, Bron, France; 3 Hospices Civils de Lyon, Lyon, France; 4 Centre Hospitalier
907
d’Arles, Arles, France; 5 Hospices Civils de Lyon, Universite´ de Lyon, CNRS UMR 5558, Lyon, France Steroids are nowadays routinely used as a long-term treatment in Duchenne muscular dystrophy (DMD). Their effects on body composition were assessed using Dual X-ray absorptiometry. The study followed over two years 29 genetically confirmed DMD patients: 21 in the steroid-treated group and 8 in the steroid-naı¨ve group. After two years of steroid treatment, the lean tissue mass values increased significantly (p < 0.0001), the percentage of body fat mass remained practically constant (p = 0.94) in comparison with the initial visit. In the steroid-naı¨ve patients, there were no significant increases in the lean tissue mass but deterioration in body composition confirmed by a significant increases in the percentage of body fat mass. Besides, significant negative correlations were found between the percentage of body fat mass and the MFM total score (R = 0.79, n = 76, p < 0.0001). A 2-year steroid treatment improves significantly body composition of boys with DMD through a significant increase in lean tissue mass. We suggest that a thorough check of body composition should be carried out before steroid treatment discontinuation in case of overweight gain. http://dx.doi:10.1016/j.nmd.2014.06.375
T.P.45 A new index of muscle development and disease progression for the pediatric patients with Duchenne muscular dystrophy, using bioelectrical impedance analysis: An observation study T. Uchiyama 1, A. Hattori 2, T. Sato 3, T. Murakami 3, K. Ishigaki 3, T. Nakayama 4, S. Kuru 5 1 TANITA Body Weight Scientific Institute, Tokyo, Japan; 2 Nagoya City University, Nagoya, Japan; 3 Tokyo Women’s Medical University, Tokyo, Japan; 4 Yokohama Rosai Hospital, Yokohama, Japan; 5 Suzuka National Hospital, Suzuka, Japan Twenty-three patients with DMD aged 3.3–27 were enrolled in our study, with the consent from parents or themselves. An impedance analyzer (BCA-100_CTanita Corp., Japan) and 10 mm wide stainless steel electrodes were used. The current used in the measurement was lower than 100 lA, and the 9 frequencies between 5 and 250 kHz. The impedance was measured at the middle part of the thigh, lower leg and upper arm. We measured the impedance index at 50 kHz (ZI50), which is generally used for estimating muscle volume in body composition analysis. We evaluated the ratio of ZI50 to the length between the electrodes, which we called net muscle volume by BIA (nMV_BIA), and MDI. The MDI is calculated by the subtraction of the division ration of the impedance at 5 kHz (Z5) by that at 250 kHz (Z250) from 1 (1 – Z5/ Z250), that was considered to reflect the ratio of intracellular volume to whole muscle bundle of extremities. The results from nMV_BIA and MDI were analysed among the ages using Spearman rank-correlation analysis. The nMV_BIA of thighs, lower legs, and upper arm were measured as 0.236, 0.160 and 0.101 cm/X, respectively. The MDI of thighs, lower legs, and upper arm were measured as 0.074, 0.121 and 0.093, respectively. The nMV_BIA had no relationships with the age of the patients, however, the MDI significantly decreased with the ages contrarily (p < 0.001). We showed the decreased MDI with progression of the disease, and it suggests that we will be able to evaluate dystrophic change of muscle simultaneously using BIA. We consider that this new method is suitable for the biomarker of the disease progression and therapies for the patients with DMD. http://dx.doi:10.1016/j.nmd.2014.06.376
T.P.43 Effects of proximal and distal muscle weakness on performance in neuromuscular diseases I. Alemdaroglu 1, P. Kaya 2, O. Yilmaz 2, A.A. Karaduman 2, H. Topaloglu 2 1 Bezmy Alem University, Istanbul, Turkey; 2 Hacettepe University, Ankara, Turkey The aim of this study was to investigate and compare the performance exposure due to different muscle weakness involvement in proximally and distally affected neuromuscular diseases (NMD’s). A total of 40 pediatric patients with different NMD’s who were grouped based on proximal (Group 1) and distal (Group 2) muscle weakness involvement were included in the study. The children between Grade 1 and 3 according to Brooke Lower Extremity Functional Classification (BLEFC) were taken under assessments. Lower extremity regional and total muscular strengths were assessed with manual muscle testing. 10 m walking time (s) and 6 Minute Walk Test (6MWT) (m) were used to evaluate performance level. The relationship between muscle weakness and performance levels was analyzed. 20 patients with proximal (Duchenne Muscular Dystrophy-DMD) and 20 with distal muscle weakness (Hereditary Sensory Motor Neuropathy) who were respectively 9 ± 3.1 and 12.9 ± 3.3 years old were included in the study. Lower extremity total muscle strengths were statistically different with 81.5 ± 18.7 in group 1 and 98.7 ± 16.5 in group 2 (z = 2.764; p < 0.05). There were not any differences in 10 m walking time (z = 0.122) and 6MWT distances (z = 0.609) between two groups (p > 0.05). There were positive correlations between lower extremity muscles strength scores except for ankle girdle and 6MWT distance (p < 0.01); negative, moderate correlations between 10 m walking time and regional/total lower extremity strength scores in group 1 (p < 0.01). There was not any relationship between regional/total muscular strengths and performance tests in Group 2 (p > 0.05). Muscle weakness distribution is an important performance determinative in NMD’s. Performance is more effected in proximal muscle weakness involvement than distal at early stages of the disease. http://dx.doi:10.1016/j.nmd.2014.06.374
T.P.44 Influence of a two-year steroid treatment on body composition as measured by Dual X-ray Absorptiometry in boys with Duchenne Muscular Dystrophy C. Vuillerot 1, P. Braillon 2, S. Fontaine-Carbonnel 2, P. Rippert 3, E. Andre 4, J. Iwaz 5, I. Poirot 2, C. Berard 2 1 Hospices Civils de Lyon, Universite´ de Lyon, CNRS UMR 5558, Bron, France; 2 Hospices Civils de Lyon, Hospital Femme Mere Enfant, Bron, France; 3 Hospices Civils de Lyon, Lyon, France; 4 Centre Hospitalier
907
d’Arles, Arles, France; 5 Hospices Civils de Lyon, Universite´ de Lyon, CNRS UMR 5558, Lyon, France Steroids are nowadays routinely used as a long-term treatment in Duchenne muscular dystrophy (DMD). Their effects on body composition were assessed using Dual X-ray absorptiometry. The study followed over two years 29 genetically confirmed DMD patients: 21 in the steroid-treated group and 8 in the steroid-naı¨ve group. After two years of steroid treatment, the lean tissue mass values increased significantly (p < 0.0001), the percentage of body fat mass remained practically constant (p = 0.94) in comparison with the initial visit. In the steroid-naı¨ve patients, there were no significant increases in the lean tissue mass but deterioration in body composition confirmed by a significant increases in the percentage of body fat mass. Besides, significant negative correlations were found between the percentage of body fat mass and the MFM total score (R = 0.79, n = 76, p < 0.0001). A 2-year steroid treatment improves significantly body composition of boys with DMD through a significant increase in lean tissue mass. We suggest that a thorough check of body composition should be carried out before steroid treatment discontinuation in case of overweight gain. http://dx.doi:10.1016/j.nmd.2014.06.375
T.P.45 A new index of muscle development and disease progression for the pediatric patients with Duchenne muscular dystrophy, using bioelectrical impedance analysis: An observation study T. Uchiyama 1, A. Hattori 2, T. Sato 3, T. Murakami 3, K. Ishigaki 3, T. Nakayama 4, S. Kuru 5 1 TANITA Body Weight Scientific Institute, Tokyo, Japan; 2 Nagoya City University, Nagoya, Japan; 3 Tokyo Women’s Medical University, Tokyo, Japan; 4 Yokohama Rosai Hospital, Yokohama, Japan; 5 Suzuka National Hospital, Suzuka, Japan Twenty-three patients with DMD aged 3.3–27 were enrolled in our study, with the consent from parents or themselves. An impedance analyzer (BCA-100_CTanita Corp., Japan) and 10 mm wide stainless steel electrodes were used. The current used in the measurement was lower than 100 lA, and the 9 frequencies between 5 and 250 kHz. The impedance was measured at the middle part of the thigh, lower leg and upper arm. We measured the impedance index at 50 kHz (ZI50), which is generally used for estimating muscle volume in body composition analysis. We evaluated the ratio of ZI50 to the length between the electrodes, which we called net muscle volume by BIA (nMV_BIA), and MDI. The MDI is calculated by the subtraction of the division ration of the impedance at 5 kHz (Z5) by that at 250 kHz (Z250) from 1 (1 – Z5/ Z250), that was considered to reflect the ratio of intracellular volume to whole muscle bundle of extremities. The results from nMV_BIA and MDI were analysed among the ages using Spearman rank-correlation analysis. The nMV_BIA of thighs, lower legs, and upper arm were measured as 0.236, 0.160 and 0.101 cm/X, respectively. The MDI of thighs, lower legs, and upper arm were measured as 0.074, 0.121 and 0.093, respectively. The nMV_BIA had no relationships with the age of the patients, however, the MDI significantly decreased with the ages contrarily (p < 0.001). We showed the decreased MDI with progression of the disease, and it suggests that we will be able to evaluate dystrophic change of muscle simultaneously using BIA. We consider that this new method is suitable for the biomarker of the disease progression and therapies for the patients with DMD. http://dx.doi:10.1016/j.nmd.2014.06.376