A standardized method for the evaluation of respiratory muscle endurance in patients with Duchenne muscular dystrophy

Matecki, Stéfan; Topin, Nathalie; Hayot, Maurice; Rivier, François; Échenne, B.; Préfaut, Christian; Ramonatxo, M

doi:10.1016/s0960-8966(00)00179-6

Cited by 44 publications

(43 citation statements)

References 28 publications

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“…Consistent with a slower phenotype was the smaller relative force decrement and improved recovery of force after repeated contractile activity in EDL, soleus, and diaphragm muscles of mdx CnA␣* compared with mdx mice. Impaired muscle endurance is characteristic of many neuromuscular disorders (12), and boys with DMD are especially susceptible to respiratory muscle fatigue (28). Since slow-twitch muscles are resistant to fatigue compared with fast muscles, a shift to a slower muscle phenotype may have therapeutic benefits for DMD patients.…”

Section: Discussionmentioning

confidence: 99%

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Stimulation of calcineurin Aα activity attenuates muscle pathophysiology inmdxdystrophic mice

Stupka¹,

Schertzer²,

Bassel‐Duby³

et al. 2008

American Journal of Physiology-Regulatory, Integrative and Comp

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Calcineurin activation ameliorates the dystrophic pathology of hindlimb muscles in mdx mice and decreases their susceptibility to contraction damage. In mdx mice, the diaphragm is more severely affected than hindlimb muscles and more representative of Duchenne muscular dystrophy. The constitutively active calcineurin Aalpha transgene (CnAalpha) was overexpressed in skeletal muscles of mdx (mdx CnAalpha*) mice to test whether muscle morphology and function would be improved. Contractile function of diaphragm strips and extensor digitorum longus and soleus muscles from adult mdx CnAalpha* and mdx mice was examined in vitro. Hindlimb muscles from mdx CnAalpha* mice had a prolonged twitch time course and were more resistant to fatigue. Because of a slower phenotype and a decrease in fiber cross-sectional area, normalized force was lower in fast- and slow-twitch muscles of mdx CnAalpha* than mdx mice. In the diaphragm, despite a slower phenotype and a approximately 35% reduction in fiber size, normalized force was preserved. This was likely mediated by the reduction in the area of the diaphragm undergoing degeneration (i.e., mononuclear cell and connective and adipose tissue infiltration). The proportion of centrally nucleated fibers was reduced in mdx CnAalpha* compared with mdx mice, indicative of improved myofiber viability. In hindlimb muscles of mdx mice, calcineurin activation increased expression of markers of regeneration, particularly developmental myosin heavy chain isoform and myocyte enhancer factor 2A. Thus activation of the calcineurin signal transduction pathway has potential to ameliorate the mdx pathophysiology, especially in the diaphragm, through its effects on muscle degeneration and regeneration and endurance capacity.

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Section: Discussionmentioning

confidence: 99%

“…As a consequence of the progressive muscle degeneration, exercise performance is limited in boys with DMD, even in the early stages of the disease. Endurance capacity and muscle strength are lower in boys with DMD than in age-matched controls (45), and their respiratory muscles are more susceptible to fatigue (28).…”

mentioning

confidence: 91%

Stimulation of calcineurin Aα activity attenuates muscle pathophysiology inmdxdystrophic mice

Stupka¹,

Schertzer²,

Bassel‐Duby³

et al. 2008

American Journal of Physiology-Regulatory, Integrative and Comp

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“…Furthermore, respiratory function impairment is recognized as a key predictor of mortality in DMD patients. 1,14 Therefore, the enhanced resistance to fatigue during regular activation of the diaphragm muscles of dystrophic mice treated with IGF-I represents a significant physiological benefit of this intervention with potential application to conditions such as DMD in which respiratory muscle function is compromised. 1,14 These data also indicate that some features of the muscle pathology of this model are affected by the intervention more than others.…”

Section: Discussionmentioning

confidence: 99%

“…1,14 Therefore, the enhanced resistance to fatigue during regular activation of the diaphragm muscles of dystrophic mice treated with IGF-I represents a significant physiological benefit of this intervention with potential application to conditions such as DMD in which respiratory muscle function is compromised. 1,14 These data also indicate that some features of the muscle pathology of this model are affected by the intervention more than others. This idea is consistent with the concept that IGF-I can influence various features of skeletal muscle development and adaptation through several different mechanisms, some of which may modulate the structural and functional characteristics of dystrophic skeletal muscles more than others.…”

Section: Discussionmentioning

confidence: 99%

Improved Contractile Function of the mdx Dystrophic Mouse Diaphragm Muscle after Insulin-Like Growth Factor-I Administration

Gregorevic

Plant

Leeding

et al. 2002

The American Journal of Pathology

108

102

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Limited knowledge exists regarding the efficacy of insulin-like growth factor I (IGF-I) administration as a therapeutic intervention for muscular dystrophies, although findings from other muscle pathology models suggest clinical potential. The diaphragm muscles of mdx mice (a model for Duchenne muscular dystrophy) were examined after 8 weeks of IGF-I administration (1 mg/kg s.c.) to test the hypothesis that IGF-I would improve the functional properties of dystrophic skeletal muscles. Force per cross-sectional area was ϳ49% greater in the muscles of treated mdx mice (149.6 ؎ 9.6 kN/m 2 ) compared with untreated mice (100.1 ؎ 4.6 kN/m 2 , P < 0.05), and maintenance of force over repeated maximal contraction was enhanced ϳ30% in muscles of treated mice (P < 0.05). Diaphragm muscles from treated mice comprised fibers with ϳ36% elevated activity of the oxidative enzyme succinate dehydrogenase, and ϳ23% reduction in the proportion of fast IId/x muscle fibers with concomitant increase in the proportion of type IIa fibers compared with untreated mice (P < 0.05). The data demonstrate that IGF-I administration can enhance the fatigue resistance of respiratory muscles in an animal model of dystrophin deficiency, in conjunction with enhancing energenic enzyme activity. As respiratory function is a mortality predictor in Duchenne muscular dystrophy patients, further evaluation of IGF-I intervention is recommended. (Am J

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“…7 The tension-time index of the diaphragm is closely related to the endurance time of the diaphragm (T lim ) and well established as a predictor of the conditions under which the diaphragm will develop output failure because of muscular fatigue. [7][8][9] Because this technique requires the use of esophageal and gastric balloons, it is too invasive to be applied in children and is not well suited for routine clinical assessment of larger patient groups. 10,11 Furthermore, it gives no information about the function of other inspiratory muscles that may be distinctly altered in different disease states, including NMD.…”

mentioning

confidence: 99%

Noninvasive Determination of the Tension-Time Index in Duchenne Muscular Dystrophy

Hahn

Duisberg

Neubauer

et al. 2009

American Journal of Physical Medicine &Amp; Rehabilitation

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A standardized method for the evaluation of respiratory muscle endurance in patients with Duchenne muscular dystrophy

Cited by 44 publications

References 28 publications

Stimulation of calcineurin Aα activity attenuates muscle pathophysiology inmdxdystrophic mice

Stimulation of calcineurin Aα activity attenuates muscle pathophysiology inmdxdystrophic mice

Improved Contractile Function of the mdx Dystrophic Mouse Diaphragm Muscle after Insulin-Like Growth Factor-I Administration

Noninvasive Determination of the Tension-Time Index in Duchenne Muscular Dystrophy

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