Noninvasive assessment of respiratory muscle strength and activity in Myotonic dystrophy

PLoS One. 2017 Jun 8;12(6):e0177318. doi: 10.1371/journal.pone.0177318. eCollection 2017.

Abstract

Objective: To evaluate sensitivity/specificity of the maximum relaxation rate (MRR) of inspiratory muscles, amplitude of electromyographic activity of the sternocleidomastoid (SCM), scalene (SCA), parasternal (2ndIS) and rectus abdominis (RA) muscles; lung function and respiratory muscle strength in subjects with Myotonic dystrophy type 1 (DM1) compared with healthy subjects.

Design and methods: Quasi-experimental observational study with control group. MRR of inspiratory muscles, lung function and amplitude of the electromyographic activity of SCM, SCA, 2ndIS and RA muscles during maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) and sniff nasal inspiratory pressure (SNIP) tests were assessed in eighteen DM1 subjects and eleven healthy.

Results: MRR was lower in DM1 group compared to healthy (P = 0.001) and was considered sensitive and specific to identify disease in DM1 and discard it in controls, as well as SNIP% (P = 0.0026), PImax% (P = 0.0077) and PEmax% (P = 0.0002). Contraction time of SCM and SCA was higher in DM1 compared to controls, respectively, during PImax (P = 0.023 and P = 0.017) and SNIP (P = 0.015 and P = .0004). The DM1 group showed lower PImax (P = .0006), PEmax (P = 0.0002), SNIP (P = 0.0014), and higher electromyographic activity of the SCM (P = 0.002) and SCA (P = 0.004) at rest; of 2ndIS (P = 0.003) during PEmax and of SCM (P = 0.02) and SCA (P = 0.03) during SNIP test.

Conclusions: MD1 subjects presented restrictive pattern, reduced respiratory muscle strength, muscular electrical activity and MRR when compared to higher compared to controls. In addition, the lower MRR found in MD1 subjects showed to be reliable to sensitivity and specificity in identifying the delayed relaxation of respiratory muscles.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Anthropometry
  • Electromyography
  • Exhalation / physiology
  • Female
  • Humans
  • Inhalation / physiology
  • Male
  • Muscle Relaxation / physiology
  • Muscle Strength / physiology*
  • Myotonic Dystrophy / physiopathology*
  • Pressure
  • ROC Curve
  • Respiratory Function Tests
  • Respiratory Muscles / physiopathology*
  • Sample Size
  • Time Factors

Grants and funding

The study received financial support of Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES), PROCAD 2013 88881.068409/2014-01, Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) processo: 400316/2012-9. Guilherme Augusto de Freitas Fregonezi is fellow from CNPq number 307353/2015-0 and Vanessa Resqueti is fellow from CNPq number 310091/2015-2. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.