Spinal bracing and lung function in type-2 spinal muscular atrophy

Pede, Chiara Di; Salamon, Eleonora; Motta, Matteo; Agosto, Caterina; Benini, Franca; Ferrari, Adriano

doi:10.23736/s1973-9087.18.05046-3

Cited by 3 publications

(3 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These interventions introduced confounding effects in data analysis. Specifically, bracing caused back muscle weakness [18,27] and exercise improved muscle endurance [28][29][30][31], both of which can affect paraspinal muscle EMG activity. Thus, these studies could not reveal the effect of paraspinal muscle activity on the natural course of curve progression.…”

Section: Discussionmentioning

confidence: 99%

Electromyographic Discrepancy in Paravertebral Muscle Activity Predicts Early Curve Progression of Untreated Adolescent Idiopathic Scoliosis

Fan,

To,

Yeung

et al. 2023

Asian Spine J

View full text Add to dashboard Cite

Study Design: This study adopted a prospective cohort study design.Purpose: This study aimed to examine electromyogram (EMG) discrepancy in paravertebral muscle activity and scoliosis progression, determine how vertebral morphology and EMG discrepancy evolve during scoliosis progression, and identify differences in EMG activity between individuals with and without adolescent idiopathic scoliosis (AIS).Overview of Literature: Higher EMG activity is observed in the convex side of scoliotic curves, but not in populations without scoliosis, suggesting that higher EMG activity is a causative factor for curve progression.Methods: In this study, 267 matched pairs of AIS and controls were recruited. The participants underwent EMG measurements at their first presentation and did not receive any treatment for 6 months at which point they underwent EMG and radiographs. Early curve progression was defined as >5° in Cobb angle at 6 months. The root mean square of the EMG (rms-EMG) signal was recorded with the participants in sitting and back extension. The rms-EMG ratio at the upper end vertebrae, apical vertebrae (AV), and lower end vertebrae (LEV) of the major curve was calculated.Results: The rms-EMG ratio in the scoliosis cohort was high compared with that in the controls (sitting: 1.2±0.3 vs. 1.0±0.1, p<0.01; back extension: 1.1±0.2 vs. 1.0±0.1, p<0.01). An AV rms-EMG ratio in back extension, with a cutoff threshold of ≥1.5 in the major thoracic curve and ≥1.3 in the major lumbar curve, was a risk factor for early curve progression after 6 months without treatment (odds ratio, 4.1; 95% confidence interval, 2.8–5.9; p<0.01). Increases in side deviation (SD) (distance between the AV and the central sacral line) were related to a higher rms-EMG ratio in LEV of the major thoracic curve (baseline: rs=0.2, p=0.03; 6 months: rs=0.3, p<0.01).Conclusions: An EMG discrepancy was detected in the scoliosis cohort, which was related to increases in SD in the major thoracic curve. The AV rms-EMG ratio in back extension was correlated with curve progression after 6 months of no treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

Electromyographic Discrepancy in Paravertebral Muscle Activity Predicts Early Curve Progression of Untreated Adolescent Idiopathic Scoliosis

Fan,

To,

Yeung

et al. 2023

Asian Spine J

View full text Add to dashboard Cite

show abstract

“…In neuromuscular disorders, spinal bracing improves postural control in sitting position and supports upper limb functionality. 37 Some studies have analyzed the consequences of spinal bracing on respiratory function in neuromuscular diseases and their results showed a statistically significant reduction of forced vital capacity in pulmonary function tests performed while wearing a brace. 38,39,40 For this reason, braces can be used, provided that they do not compromise pulmonary function.…”

Section: Orthoses and Adaptative Equipmentmentioning

confidence: 99%

Rehabilitation approach to spinal muscular atrophy

et al. 2023

View full text Add to dashboard Cite

Spinal muscular atrophy is a genetically inherited severe neuromuscular disease due to degeneration of alpha motor neurons in spinal cord and brainstem. SMA is divided into clinical subtypes using the age of onset and achieved development milestones. It is characterized by tetraparesis with a proximal pattern, affecting predominately the lower limbs and hypotonia. Recently, the paradigm of SMA has changed after approval of the first disease-modifying therapy. The pharmacological treatment will have a better outcome if accompanied by a structured rehabilitation care program. In this review, we discuss the management of feeding and swallowing disorders, musculoskeletal deformities, sleep disorders and pulmonary impairment. We will review the therapeutic approaches, including positioning, stretching and exercise, as well as assistive devices prescription.

show abstract

“…In the Veneto region of Italy, children diagnosed with SMA are cared for from birth in the pediatric palliative care (PPC) program, which provides coordination of follow-up, enrollment therapy, parental qualification and network coordination (15). In the referral PPC Centre of Padua (Veneto), during the first lockdown period, some activities had to be changed or suspended.…”

Section: Introductionmentioning

confidence: 99%

Effect of the COVID-19 Pandemic on Children With SMA Receiving Nusinersen: What Is Missed and What Is Gained?

Agosto

Salamon

Giacomelli³

et al. 2021

Front. Neurol.

Self Cite

View full text Add to dashboard Cite

Nusinersen is the first oligonucleotide-based drug that is approved for the treatment of spinal muscular atrophy. In January 2020, the WHO declared COVID-19 a pandemic and nusinersen-provider centers had to postpone planned infusions for some children along with other related interventions. Considering the important contribution that the intrathecal infusions and other support activities could have on the quality of life of spinal muscular atrophy patients and their families, this emergency could have a relevant impact on the course of the pathology. The present work aims to assess the clinical and social issues that arise for spinal muscular atrophy children in care at the referral pediatric palliative care Centre of Padua (Veneto) from a delay in nusinersen infusions, resulting from the contingent COVID-19 restrictions. This evaluation has been carried out in both the short and long term after the first lockdown period and can be considered as a “proxy” of a situation of a possible delay in administration or management of infusions, due to other different causes.

show abstract

Spinal bracing and lung function in type-2 spinal muscular atrophy

Cited by 3 publications

References 8 publications

Electromyographic Discrepancy in Paravertebral Muscle Activity Predicts Early Curve Progression of Untreated Adolescent Idiopathic Scoliosis

Electromyographic Discrepancy in Paravertebral Muscle Activity Predicts Early Curve Progression of Untreated Adolescent Idiopathic Scoliosis

Rehabilitation approach to spinal muscular atrophy

Effect of the COVID-19 Pandemic on Children With SMA Receiving Nusinersen: What Is Missed and What Is Gained?

Contact Info

Product

Resources

About