scholarly journals Clinical Variability in Spinal Muscular Atrophy Type III

2020 ◽  
Vol 88 (6) ◽  
pp. 1109-1117
Author(s):  
Giorgia Coratti ◽  
Sonia Messina ◽  
Simona Lucibello ◽  
Maria Carmela Pera ◽  
Jacqueline Montes ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type ◽  
Clinical Variability ◽  
Type Iii

Reversal of neuromuscular blockade with sugammadex in a patient with spinal muscular atrophy type III (Kugelberg–Welander syndrome)

2011 ◽  
Vol 26 (2) ◽  
pp. 306-307 ◽  
Author(s):  
Hugo Vilela ◽  
João Santos ◽  
João Colaço ◽  
Eduardo Oliveira ◽  
Pedro Canas-da-Silva
Keyword(s):  
Spinal Muscular Atrophy ◽  
Neuromuscular Blockade ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii

P16.4 Response to daily salbutamol in spinal muscular atrophy type III

2011 ◽  
Vol 15 ◽  
pp. S96 ◽  
Author(s):  
G. McCullagh ◽  
M. Main ◽  
F. Muntoni ◽  
A.Y. Manzur ◽  
M.-C. Scoto ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii

scholarly journals Influence of hybrid assistive limb gait training on spatial muscle activation patterns in spinal muscular atrophy type III

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 214
Author(s):  
Yuichi Nishikawa ◽  
Kohei Watanabe ◽  
Naoya Orita ◽  
Noriaki Maeda ◽  
Hiroaki Kimura ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscle Activation ◽  
Muscular Atrophy ◽  
Training Session ◽  
Gait Training ◽  
Post Intervention ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii ◽  
Hybrid Assistive Limb ◽  
Modified Entropy

Background: Despite the potential benefits, the effects of Hybrid Assistive Limb (HAL) gait training on changes in neuromuscular activation that accompany functional gains in individuals with spinal muscular atrophy (SMA) type III is not well known. In this article, we quantify the effects of HAL gait training on spatial muscle activity patterns in a patient with SMA type III using multi-channel surface electromyography (SEMG). Methods: A 21-years old male (168 cm, 47.8 kg) with spinal muscular atrophy type III, when diagnosed at 18-years old by genetic screening, participated in this case study. Although he presented with forearm distal muscle weakness, atrophy of the intrinsic muscles of the hand, and neuromuscular fatigue, his activities of daily living is independent. The patient underwent a separate, single 33-minute session of both HAL and treadmill gait training. To evaluate the coefficient of variation (CoV) of force and alterations in the SEMG spatial distribution patterns, modified entropy and CoV of root mean square (RMS) were calculated from the vastus lateralis (VL) muscle before and after the intervention of HAL and treadmill gait training. Each training session was separated by a period of one month to avoid cross-over effects. Results: There was a greater decrease in the ΔCoV of force and an increase in the magnitude of whole VL muscle activation from pre-intervention to post-intervention with the HAL gait training as compared to the treadmill gait training. In response to only HAL gait training, the CoV of RMS was higher, and the modified entropy was lower post-intervention than pre-intervention. Conclusions: Our results support the notion that HAL gait training has a positive benefit on motor output not only in the magnitude of SEMG generated but also the patterns of neural activation.

Spinal Muscular Atrophy Type III

2011 ◽  
Vol 27 (6) ◽  
pp. 779-785 ◽  
Author(s):  
Sally Dunaway ◽  
Jacqueline Montes ◽  
Patricia A. Ryan ◽  
Megan Montgomery ◽  
Douglas M. Sproule ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii

scholarly journals Influence of hybrid assistive limb gait training on spatial muscle activation patterns in spinal muscular atrophy type III

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 214
Author(s):  
Yuichi Nishikawa ◽  
Kohei Watanabe ◽  
Naoya Orita ◽  
Noriaki Maeda ◽  
Hiroaki Kimura ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscle Activation ◽  
Muscular Atrophy ◽  
Training Session ◽  
Gait Training ◽  
Post Intervention ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii ◽  
Hybrid Assistive Limb ◽  
Modified Entropy

Background: Despite the potential benefits, the effects of Hybrid Assistive Limb (HAL) gait training on changes in neuromuscular activation that accompany functional gains in individuals with spinal muscular atrophy (SMA) type III is not well known. In this article, we quantify the effects of HAL gait training on spatial muscle activity patterns in a patient with SMA type III using multi-channel surface electromyography (SEMG). Methods: A 21-years old male (168 cm, 47.8 kg) with spinal muscular atrophy type III, when diagnosed at 18-years old by genetic screening, participated in this case study. Although he presented with forearm distal muscle weakness, atrophy of the intrinsic muscles of the hand, and neuromuscular fatigue, his activities of daily living is independent. The patient underwent a separate, single 33-minute session of both HAL and treadmill gait training. To evaluate the coefficient of variation (CoV) of force and alterations in the SEMG spatial distribution patterns, modified entropy and CoV of root mean square (RMS) were calculated from the vastus lateralis (VL) muscle before and after the intervention of HAL and treadmill gait training. Each training session was separated by a period of one month to avoid cross-over effects. Results: There was a greater decrease in the ΔCoV of force and an increase in the magnitude of whole VL muscle activation from pre-intervention to post-intervention with the HAL gait training as compared to the treadmill gait training. In response to only HAL gait training, the CoV of RMS was higher, and the modified entropy was lower post-intervention than pre-intervention. Conclusions: Our results support the notion that HAL gait training has a positive benefit on motor output not only in the magnitude of SEMG generated but also the patterns of neural activation.

scholarly journals Exercise Combined with Electrotherapy Enhances Motor Function in an Adolescent with Spinal Muscular Atrophy Type III

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Massimiliano Gobbo ◽  
Sara Lazzarini ◽  
Laura Vacchi ◽  
Paolo Gaffurini ◽  
Luciano Bissolotti ◽  
...  
Keyword(s):  
Electrical Stimulation ◽  
Spinal Muscular Atrophy ◽  
Motor Function ◽  
Muscular Atrophy ◽  
Neuromuscular Electrical Stimulation ◽  
Stair Climbing ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii ◽  
Home Based

Background. Electrotherapy is widely used in physical therapy to increase muscle mass, improve motor function, and assist physical activity in several neurologic conditions. However, concerning Spinal Muscular Atrophy (SMA), limited evidence exists on the role of electrotherapy as an adjunct for improving muscle strength and function. Case Report. An adolescent (13 y.o.) with SMA type III underwent an 18-week strengthening program divided into two stages. During Phase I (weeks: 1-8), a home-based program for quadriceps strengthening through neuromuscular electrical stimulation (NMES) was provided. In Phase II (weeks: 9-18), at-home NMES was combined with functional electrical stimulation (FES) assisting volitional cycling for a broader, systemic conditioning. The treatment improved patient’s structural and functional motor outcomes (quadriceps circumference and strength, Tinetti scale, and Hammersmith scale) as well as independence in stair climbing. Clinical Rehabilitation Impact. The purpose of this report is to raise awareness of the potential role of electrotherapy to help improving motor performance in SMA patients and, secondly, to foster further research aimed at assessing the actual contribution this intervention may have as an add-on therapy to existing care.

Progressive Subcutaneous and Periarticular Calcifications in a Patient with Spinal Muscular Atrophy Type III

1995 ◽  
Vol 26 (06) ◽  
pp. 331-332
Author(s):  
E. Boltshauser ◽  
J. Gehrmann ◽  
M. Steinlin ◽  
G. Eich ◽  
R. Spiegel
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Spinal Muscular Atrophy Type ◽  
Type Iii
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