Predicting the loss of ambulation in Duchenne muscular dystrophy

Zanoteli, Edmar

doi:10.1590/0004-282x20130243

Cited by 8 publications

(7 citation statements)

References 8 publications

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“…The decline of the motor activities over the course of the disease is unavoidable. The use of functional assessment scales is needed for clinical follow-up, establishment of the functional diagnosis, and therapeutic decision-making 5 - 7 .…”

Section: Introductionmentioning

confidence: 99%

“…One further assessment frequently applied to individuals with DMD is the measurement of time spent in the performance of functional activities, such as climbing up stairs, standing from a seat and walking. Generally, the use of compensatory movements tends to increase the time spent in the performance of the activities tested and thus is indicative of worsening of the functional status 5 - 12 .…”

Section: Introductionmentioning

confidence: 99%

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Relationship between the climbing up and climbing down stairs domain scores on the FES-DMD, the score on the Vignos Scale, age and timed performance of functional activities in boys with Duchenne muscular dystrophy

Fernandes¹,

Caromano

Assis³

et al. 2014

Braz. J. Phys. Ther.

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BACKGROUND: Knowing the potential for and limitations of information generated using different evaluation instruments favors the development of more accurate functional diagnoses and therapeutic decision-making. OBJECTIVE: To investigate the relationship between the number of compensatory movements when climbing up and going down stairs, age, functional classification and time taken to perform a tested activity (TA) of going up and down stairs in boys with Duchenne muscular dystrophy (DMD). METHOD: A bank of movies featuring 30 boys with DMD performing functional activities was evaluated. Compensatory movements were assessed using the climbing up and going down stairs domain of the Functional Evaluation Scale for Duchenne Muscular Dystrophy (FES-DMD); age in years; functional classification using the Vignos Scale (VS), and TA using a timer. Statistical analyses were performed using the Spearman correlation test. RESULTS: There is a moderate relationship between the climbing up stairs domain of the FES-DMD and age (r=0.53, p=0.004) and strong relationships with VS (r=0.72, p=0.001) and TA for this task (r=0.83, p<0.001). There were weak relationships between the going down stairs domain of the FES-DMD-going down stairs with age (r=0.40, p=0.032), VS (r=0.65, p=0.002) and TA for this task (r=0.40, p=0.034). CONCLUSION: These findings indicate that the evaluation of compensatory movements used when climbing up stairs can provide more relevant information about the evolution of the disease, although the activity of going down stairs should be investigated, with the aim of enriching guidance and strengthening accident prevention. Data from the FES-DMD, age, VS and TA can be used in a complementary way to formulate functional diagnoses. Longitudinal studies and with broader age groups may supplement this information.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Relationship between the climbing up and climbing down stairs domain scores on the FES-DMD, the score on the Vignos Scale, age and timed performance of functional activities in boys with Duchenne muscular dystrophy

Fernandes¹,

Caromano

Assis³

et al. 2014

Braz. J. Phys. Ther.

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“…Another study [21] confirmed that loss of strength, especially from the hip extensors, as a predictor of loss of ambulation in DMD. This weakness progressively decreases the ability of a person to perform activities in standing and walking position, developing compensatory movements.…”

Section: Discussionmentioning

confidence: 77%

Decreased Gait and Function in Duchenne Muscular Dystrophy

Iwabe-Marchese¹,

Fávaro²,

Luiz³

2014

OJTR

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Duchenne muscular dystrophy (DMD) is a genetic disorder linked to chromosome Xp21, due to absence of dystrophin production. It is clinically characterized by progressive muscle weakness, fatigue, and development of joint contractures that compromise general motor functionality, mainly the gait. Objective: To characterize the motor function and decrease gait in children with DMD using the Portuguese version of the Motor Function Measure scale (MFM-P). Methods: A review of medical records including chronological age and scores from MFM-P of children with a DMD who attended at the Neuromuscular Diseases Clinic at Campinas State University (UNICAMP), Brazil was performed in this study. A total of 36 medical records of male patients with confirmed clinical diagnosis of DMD, ambulatory or not, regardless of age; excluding those with other associated diseases or other types of muscular dystrophies were selected. Data were analyzed using Kolmogorov-Smirnov and Spearman correlation statistical tests. Results: Analysis of all data collected showed that 75% of our sample had D1 scores lower than 41.02%. There was a linear relationship between the scores of D2 and D3, but no association between D2 and D1 scores was noted. D1 score was between 40% and 80% in those patients presenting D2 scores between 80% and 100%. In all cases patients with low total score presented a greater risk for loss of gait and their functionality. Conclusion: The standing posture and the postural transfers were the worst activities observed in children with DMD, with positive correlation between proximal and distal motor function. Even with high scores according MFM-P in proximal function, the children showed strong predictors for loss of gait.

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“…Finally, Brazilian investigators have broken new ground with RBD research in at least three directions: (i) Wilson׳s disease (hepatolenticular degeneration) presenting with RBD as the initial symptom [35] , which comprises the first published description of RBD in Wilson׳s disease in the literature, and adds further evidence to the parallelism of PD and Wilson׳s disease in phenotype and brainstem topography. (ii) Cannabidiol (CBD) therapy in RBD associated with PD [36] .…”

Section: Update On Rbdmentioning

confidence: 99%

The far side of sleep: Towards a deeper understanding of parasomnias and nocturnal seizures

Schenck

2014

Sleep Science

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Predicting the loss of ambulation in Duchenne muscular dystrophy

Cited by 8 publications

References 8 publications

Relationship between the climbing up and climbing down stairs domain scores on the FES-DMD, the score on the Vignos Scale, age and timed performance of functional activities in boys with Duchenne muscular dystrophy

Relationship between the climbing up and climbing down stairs domain scores on the FES-DMD, the score on the Vignos Scale, age and timed performance of functional activities in boys with Duchenne muscular dystrophy

Decreased Gait and Function in Duchenne Muscular Dystrophy

The far side of sleep: Towards a deeper understanding of parasomnias and nocturnal seizures

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