GNE-myopathy (HIBM): Upper and lower extremity muscle strength declines over time in a prospective study

2017 ◽  
Vol 27 ◽  
pp. S149-S150
Author(s):  
A. Behin ◽  
T. Mozaffar ◽  
M. Tarnopolsky ◽  
T. Gidaro ◽  
O. Pogoryelova ◽  
...  
2016 ◽  
Vol 26 ◽  
pp. S169-S170 ◽  
Author(s):  
M. Tarnopolsky ◽  
T. Mozaffar ◽  
A. Behin ◽  
T. Gidaro ◽  
O. Pogoryelova ◽  
...  

2021 ◽  
pp. 1-10
Author(s):  
Hanns Lochmüller ◽  
Anthony Behin ◽  
Ivailo Tournev ◽  
Mark Tarnopolsky ◽  
Rita Horváth ◽  
...  

BACKGROUND: GNE myopathy is a rare, autosomal recessive, muscle disease caused by mutations in GNE and is characterized by rimmed vacuoles on muscle biopsy and progressive distal to proximal muscle weakness. OBJECTIVE: Investigate the clinical presentation and progression of GNE myopathy. METHODS: The GNE Myopathy Disease Monitoring Program was an international, prospective, observational study in subjects with GNE myopathy. Muscle strength was assessed with hand-held dynamometry (HHD), with upper extremity (UE) and lower extremity (LE) composite scores reflecting upper and lower extremity muscle groups, respectively. The GNE myopathy–Functional Activity Scale (GNEM-FAS) was used to further assess impairment in mobility, upper extremity function, and self-care. RESULTS: Eighty-seven of 101 enrolled subjects completed the trial until study closer by the sponsor; 60 completed 36 months. Mean (SD) HHD UE composite score decreased from 34.3 kg (32.0) at baseline to 29.4 kg (32.6) kg at month 36 (LS mean change [95%CI]: –3.8 kg [–5.9, –1.7]; P = 0.0005). Mean (SD) HHD LE composite score decreased from 32.0 kg (34.1) at baseline to 25.5 kg (31.2) at month 36 (LS mean change [95%CI]: –4.9 [–7.7, –2.2]; P = 0.0005). GNEM-FAS scores were more severe at baseline in subjects who walked <  200 meters versus≥200 meters in 6 minutes; in both groups, GNEM-FAS total, mobility, UE, and self-care scores decreased from baseline through month 36. CONCLUSIONS: These findings demonstrate progressive decline in muscle strength in GNE myopathy and provide insight into the appropriate tools to detect clinically meaningful changes in future GNE myopathy interventional trials.


Author(s):  
Chisato Hayashi ◽  
Soshiro Ogata ◽  
Tadashi Okano ◽  
Hiromitsu Toyoda ◽  
Sonoe Mashino

Abstract Background The effects of group exercise on the physical function of community-dwelling older adults remain unclear. The changes in lower extremity muscle strength, timed up and go (TUG) time, and the motor fitness scale (MFS), over time, among older adults who expressed a willingness to participate in community-based physical exercise groups, were determined using multilevel modelling. Methods We analyzed data of 2407 older adults between April 2010 and December 2019 from the registry of physical tests of community-based physical exercise groups. We conducted a retrospective cohort study to assess the effect of physical exercise on lower extremity muscle strength, TUG time, and MFS scores. The durations of the exercises were evaluated by frequency of physical test’s participate. Results A deterioration in lower extremity muscle strength was found in the short-term participant group only. However, in the mid-term and long-term participation groups, lower extremity muscle strength showed a trend of improvement. The TUG time and the MFS score were negatively correlated with increasing age in both groups divided by the duration of participation. However, there was a slower rate of deterioration in the long-term participation group. Discussion Lower extremity muscle strength, TUG time, and MFS scores decline with increasing age and there were differences in the slope of deterioration that depended on the duration of participation in community-based group exercise. Conclusion Participation in group exercise improved lower extremity muscle strength, TUG time, and MFS scores of older adults living in a community. The positive effects of group exercise were dependent on long-term participation.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Hiroki Yabe ◽  
Yuto Imoto ◽  
Ayaka Onoyama ◽  
Sayaka Ito ◽  
Kenichi Kono ◽  
...  

Abstract Background The risk of adverse events associated with peritoneal dialysis (PD) in elderly patients has not been thoroughly investigated. The purpose of this study was to assess the association between physical function and hospitalization in elderly PD patients. Methods This is a single-center prospective observational cohort study. Thirty-three aged patients (74.8 ± 5.9 years) participated in a 6-min walk distance, short physical performance battery (SPPB), lower extremity muscle strength, and 10-m walk speed. All subjects were followed until hospitalization to the end of the follow-up period. Results The 6-min walk distance was 332 ± 112.5 m; SPPB was 11 (8.3–12) points; the lower extremity muscle strength was 36.6 ± 9.6%; 10-m walk speed was 1.1± 0.2 m/s. During the follow-up, 19 patients (57.5%) were hospitalized. In the Kaplan-Meier survival analysis and log-rank test, a lower 6-min walk distance and PD vintage were significantly associated with hospitalization (p<0.05). After adjustment for PD vintage in Cox proportional analysis, the 6-min walk distance remained associated with hospitalization (95% confidence interval, 0.98–0.99). Conclusion Lower exercise tolerance assessed by the 6-min walk distance was significantly associated with hospitalization in elderly PD patients. Our findings indicate that measurement and intervention of exercise intolerance are essential to predict the clinical outcomes of elderly PD patients. Trial registration This study was prospectively registered at inception in the UMIN Clinical Trials Registry under identification number UMIN000038405.


Nutrients ◽  
2017 ◽  
Vol 9 (4) ◽  
pp. 379 ◽  
Author(s):  
Antoneta Granic ◽  
Tom Hill ◽  
Karen Davies ◽  
Carol Jagger ◽  
Ashley Adamson ◽  
...  

2017 ◽  
Vol Volume 10 ◽  
pp. 187-194 ◽  
Author(s):  
Bokun Kim ◽  
Takehiko Tsujimoto ◽  
Rina So ◽  
Xiaoguang Zhao ◽  
Sechang Oh ◽  
...  

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