Evaluation of therapeutic electrical stimulation to improve muscle strength and function in children with types II/III spinal muscular atrophy

Author(s):  
Darcy L Fehlings ◽  
Susan Kirsch ◽  
Alan McComas ◽  
Mary Chipman ◽  
Kent Campbell
2017 ◽  
Vol 5 (2_suppl2) ◽  
pp. 2325967117S0010
Author(s):  
Sezen Karabörklü Argut ◽  
Nilgün Türker ◽  
Derya Çelik ◽  
Önder İsmet Kılıçoğlu

Objective: The weakness of the quadriceps strength in patellofemoral pain syndrome (PFPS) is very evident. Therefore, quadriceps strengthening exercises are very important part of the rehabilitation program. Neuromuscular Electrical Stimulation (NMES) is considered one of the methods for increasing quadriceps muscle strength. To evaluate the effectiveness of combined NMES and strengthening exercises to improve the recovery of quadriceps strength and function in patients with PFPS. Methods: This study was planned as a randomized controlled pilot study. A total of 27 patients (mean age=38.9±10.8 years, range=20-60 years; 16 females) with PFPS were assessed and randomly assigned into 2 groups. Group I received a standard program (quadriceps strengthening, hip strengthening and hamstring stretching) and NMES combined with quadriceps strengthening exercises simultaneously. Group II received the same standard program without NMES. Both groups were enrolled 3 times per week for 40 minutes per day in 6 weeks. Patients were assessed at the baseline, 3 rd, 6th, and 12th weeks of treatment. Quadriceps strength was evaluated by isokinetic dynamometer. The range of motion at testing was set between 0 for extension to 90 for flexion. The test was performed at 60 degrees/sn and concentric maximum peak torque value was recorded. Kujala and Lysholm scores were used for functional assessments. The data were analyzed using the SPSS 20.0. Shapiro-Wilk test was used to assess the distribution of data. The changes in dependent variables before treatment, 3 rd, 6th, and 12th weeks were analyzed using a 2 by 4 mixed-model analysis of variance (ANOVA). Pairwise comparisons with paired t test were used to determine whether the Group I or Group II, has changed over time. An intention- to- treat analysis was performed to impute values for missing data. An alpha level of 0.05 was established. Results: The study was completed with 20 patients. Group I (n=10; mean age=39.4±8.5 years; 7 females) and group II (n=10; mean age=43.2±11.7 years; 5 females) had no differences in pre-operative measures (p>.05). There was significant improvements in within groups statistics of all parameters for both groups (p<.05). No differences in quadriceps strength, Kujala and Lysholm scores between groups were found at the different time points (F= 0.86; p = 0.12, F=0,001; p =0.97, F=0.12; p=0.73, respectively) Conclusion: NMES combined with quadriceps strengthening exercises has no additional effect on PFPS patients’ on muscle strength and function. When considering these results, we believe that there is no need to continue the study in progress. [Table: see text]


Neurology ◽  
2020 ◽  
Vol 95 (14) ◽  
pp. e1988-e1998 ◽  
Author(s):  
Camiel A. Wijngaarde ◽  
Marloes Stam ◽  
Louise A.M. Otto ◽  
Bart Bartels ◽  
Fay-Lynn Asselman ◽  
...  

ObjectiveTo assess longitudinal patterns of muscle strength, motor function, and maximal compound muscle action potential amplitudes (CMAPMAX) in older patients with spinal muscular atrophy (SMA), hypothesizing a continued decline of motor function parameters throughout life.MethodsWe measured muscle strength (Medical Research Council), motor function (Hammersmith Functional Motor Scale Expanded [HFMSE] and Motor Function Measure), and CMAPMAX in treatment-naive patients. We used both longitudinal and cross-sectional data in mixed models to analyze natural history patterns.ResultsWe included 250 patients with SMA types 1c through 4. Median patient age at assessment was 26.8 years, the number of assessments per patient ranged from 1 to 6. Baseline muscle strength and motor function scores differed significantly between SMA types, but annual rates of decline were largely similar and mostly linear. HFMSE floor effects were present for all patients with SMA type 1c, and adolescents and adults with types 2 and 3a. CMAPMAX differed significantly between SMA types but did not decline significantly with increasing age. Muscle strength correlated very strongly with motor function (τ ≥ 0.8) but only moderately with CMAPMAX (τ ≈ 0.5–0.6).ConclusionMuscle strength and motor function decline in older patients with SMA are constant without periods of slower progression or a plateau phase. The floor effects of the HFMSE preclude its use for long-term follow-up of adult patients with SMA types 1c through 3a. Muscle strength sum scores represent an alternative, feasible outcome measure for adolescent and adult patients with SMA.


2018 ◽  
Vol 25 (3) ◽  
pp. 512-518 ◽  
Author(s):  
R. I. Wadman ◽  
C. A. Wijngaarde ◽  
M. Stam ◽  
B. Bartels ◽  
L. A. M. Otto ◽  
...  

2007 ◽  
Vol 179 (1) ◽  
pp. 139-149 ◽  
Author(s):  
Sibylle Jablonka ◽  
Marcus Beck ◽  
Barbara Dorothea Lechner ◽  
Christine Mayer ◽  
Michael Sendtner

Proximal spinal muscular atrophy (SMA) is a motoneuron disease for which there is currently no effective treatment. In animal models of SMA, spinal motoneurons exhibit reduced axon elongation and growth cone size. These defects correlate with reduced β-actin messenger RNA and protein levels in distal axons. We show that survival motoneuron gene (Smn)–deficient motoneurons exhibit severe defects in clustering Cav2.2 channels in axonal growth cones. These defects also correlate with a reduced frequency of local Ca2+ transients. In contrast, global spontaneous excitability measured in cell bodies and proximal axons is not reduced. Stimulation of Smn production from the transgenic SMN2 gene by cyclic adenosine monophosphate restores Cav2.2 accumulation and excitability. This may lead to the development of new therapies for SMA that are not focused on enhancing motoneuron survival but instead investigate restoration of growth cone excitability and function.


2013 ◽  
Vol 17 (6) ◽  
pp. 552-560 ◽  
Author(s):  
Sonia Khirani ◽  
Marina Colella ◽  
Valeria Caldarelli ◽  
Guillaume Aubertin ◽  
Michèle Boulé ◽  
...  

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