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Related Concept Videos

Alterations in Muscle Tone ll01:12

Alterations in Muscle Tone ll

31
Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
31
Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

36
Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
36

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Longitudinal Changes of Motor Function in Becker Muscular Dystrophy.

Luca Bello1, Pietro Riguzzi1, Giuliana Capece1

  • 1Department of Neuroscience DNS, University of Padova, Italy; and.

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|July 30, 2025
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Summary

Becker muscular dystrophy (BMD) shows slow progression, with most patients retaining ambulation past 60. Specific genetic variants influence disease trajectory and functional decline, aiding clinical trial design.

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Area of Science:

  • Neurology
  • Genetics
  • Clinical Research

Background:

  • Becker muscular dystrophy (BMD) arises from Duchenne muscular dystrophy gene variants causing partial dystrophin expression.
  • Understanding disease progression and predictive factors is crucial for clinical trials.

Purpose of the Study:

  • To describe disease trajectories in different genetic subgroups of BMD.
  • To identify factors predicting progressive versus stable disease.
  • To inform clinical trial design and interpretation.

Main Methods:

  • Observational longitudinal study of 107 male participants with molecularly confirmed BMD.
  • Functional evaluations included North Star Ambulatory Assessment (NSAA), 6-minute walk test, and timed function tests.
  • Follow-up involved an average of 6.4 evaluations over 6.1 years.

Main Results:

  • Only 25% of individuals with BMD lost ambulation by age 60.
  • Deletions del 45-47 and del 45-48 showed poorer function compared to del 48 and deletions ending on exon 51.
  • All measures declined over time, with faster decline in more severe genetic groups and lower baseline NSAA scores.

Conclusions:

  • Genotype-phenotype correlations in BMD are refined.
  • Quantified motor outcome measure decline can power clinical trial calculations.
  • Identified factors can guide trial inclusion/exclusion criteria and serve as comparators for real-world data.