Limb-Girdle Syndromes

McDonald, Craig; Johnson, Ernest H.; Abresch, Richard T.; Carter, G. T.; Fowler, William M.; Kilmer, David D.

doi:10.1097/00002060-199509001-00006

Cited by 59 publications

(11 citation statements)

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“…On the other hand, McDonald et al 62 and Stübgen et al 63 found a progressive decline in muscle strength and ability in LGMD individuals, but it happened slowly per decade of disease duration. Thus, despite muscular weakness, damage to muscle fibers, and movement difficulty, characterizing LGMD, the individuals in this study still have the capacity to improve performance, even to match the value with the EG.…”

Section: Discussionmentioning

confidence: 98%

Motor learning through a non-immersive virtual task in people with limb-girdle muscular dystrophies

Prumes

Silva

Alberissi

et al. 2020

jhgd

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Introduction: Limb-girdle muscular dystrophies (LGMDs) are neuromuscular and genetic disorders that progress with weakness and damage of the proximal muscles, developing with loss of functionality. Virtual reality environments are suggested as an effective alternative for performance of daily life activities. However, there is no evidence in the literature on the use of virtual reality in this population. Objective: Assess motor performance through a motor learning protocol in a coincident timing task. Methods: 10 participants with LGMD and 10 healthy individuals were selected and included in the study to perform a non-immersive virtual reality task divided into three phases: acquisition (20 attempts), retention (5 attempts), and transfer (5 attempts, with speed increase). Results: It is observed that the accuracy of movement improves from the beginning to the end of the acquisition (p = 0.01); however, there is a marginal difference between the groups in block A1 (p = 0.089). Regarding the variability of touches, observed by the variable error, both groups improved performance in all phases. Conclusion: Even with lower performance than the control group at the beginning of the practice, individuals with LGMD showed the potential to optimize motor function during the practice of a non-immersive virtual reality activity and were able to match their performance with the control group after a few attempts.

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Section: Discussionmentioning

confidence: 98%

Motor learning through a non-immersive virtual task in people with limb-girdle muscular dystrophies

Prumes

Silva

Alberissi

et al. 2020

jhgd

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“…Nine articles describing UE impairments and/or activity limitations in patients with LGMD were identified by our search strategy (Table 2). Each article described a prospective or crosssectional cohort study, with a total of 270 patients, including 123 men and 81 women (one study including 66 patients did not report gender [25]). The mean age of the included patients was 35 to 55 years (with a range of 12-81 years), and the mean disease duration was 17 to 34 years.…”

Section: Studies and Patients Includedmentioning

confidence: 99%

“…The mean age of the included patients was 35 to 55 years (with a range of 12-81 years), and the mean disease duration was 17 to 34 years. The age of onset also varied widely and was dependent on the type of LGMD [25].…”

Section: Studies and Patients Includedmentioning

confidence: 99%

“…Eight of the studies reported outcome measures at the level of UE function [17,14,[23][24][25][27][28][29], eight studies measured UE mobility activities [17,19,[23][24][25][27][28][29], and five studies included measures of self-care and domestic life [19,23,[27][28][29]. Four of the studies collected follow-up measurements [24,25,28,29].…”

Section: Outcome Measuresmentioning

confidence: 99%

“…To evaluate UE function, seven studies manually tested 18-36 muscles [17,[23][24][25][27][28][29], and two studies assessed maximal isometric and isokinetic muscle strength by measuring MVC [14,25]. One study measured grip strength and pinch strength [25], and another study performed physical examinations to evaluate posture and stability of the scapula [24]. Another study recorded half-fatigue time, which is defined as the time spent between maximum torque and half-maximum torque [14].…”

Section: Outcome Measuresmentioning

confidence: 99%

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Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review

Bergsma

Cup²,

Geurts³

et al. 2014

Disability and Rehabilitation

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Scoliosis Surgery in Children With Neuromuscular Disease

Barsdorf¹,

Sproule²,

Kaufmann³

2010

Arch Neurol

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Background: Scoliosis is a frequent complication of pediatric neuromuscular disease (NMD). Scoliosis surgery in children with NMD is thought to carry greater morbidity and mortality. Objectives: To study demographics, comorbidities, outcomes, and hospitalization expenditures among children with NMD undergoing scoliosis surgery. Design: Using the Kids Inpatient Database, a large allpayer US database of hospital discharges among children and adults younger than 20 years, we studied children undergoing scoliosis surgery between January 1, 1997, and December 31, 2003. Continuous variables were compared by t test, and categorical variables were compared by Pearson product moment correlation 2 test. Setting: National database of pediatric hospital discharges. Patients: Children with and without NMD. Main Outcome Measures: Demographics, hospital length of stay, and in-hospital mortality associated with scoliosis surgery. Results: Of 17 780 reported hospitalizations owing to scoliosis surgery, 437 children (2.5%) had NMD. Compared with children undergoing scoliosis surgery for other indications, children with NMD were more likely to be younger (12.4 vs 14.2 years), male (73.5% vs 38.3%), and insured by Medicaid (35.6% vs 20.3%). Comorbidities that were more common among children with NMD included pulmonary complications (lung disease not classified, pulmonary collapse, pulmonary insufficiency, chronic respiratory failure, and ventilator requirement) and cardiovascular complications (cardiomyopathy, hypotension, and tachycardia). Scoliosis surgery in children with NMD was associated with increased hospital length of stay (10.3 vs 7.7 days) and hospitalization expenditures ($80 251 vs $62 154), and higher in-hospital mortality (1.6% vs 0.2%). Conclusion: Children with NMD have increased hospital length of stay and higher in-hospital mortality associated with scoliosis surgery, highlighting the need for further study of measures that could reduce complications and improve outcomes in this population.

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Limb-Girdle Syndromes

Cited by 59 publications

References 0 publications

Motor learning through a non-immersive virtual task in people with limb-girdle muscular dystrophies

Motor learning through a non-immersive virtual task in people with limb-girdle muscular dystrophies

Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review

Scoliosis Surgery in Children With Neuromuscular Disease

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