6533b7d0fe1ef96bd125ba37

RESEARCH PRODUCT

Noninvasive assessment of respiratory muscle strength and activity in Myotonic dystrophy

Antonio SarmentoGuilherme FregoneziFernando Augusto Lavezzo DiasGeorge Carlos Do NascimentoAndrea AlivertiVanessa ResquetiMorgana De Araújo EvangelistaLucien Peroni GualdiMário Emílio Teixeira Dourado Júnior

subject

MaleMuscle PhysiologyTime FactorsMuscle FunctionsPhysiologyMuscle RelaxationRespiratory Systemlcsh:MedicineElectromyographyPulmonary function testing0302 clinical medicineThoracic DiaphragmMedicine and Health SciencesMyotonic DystrophyMedicineRespiratory systemlcsh:ScienceMusculoskeletal SystemAbdominal MusclesMultidisciplinaryAnthropometrymedicine.diagnostic_testMusclesMuscle AnalysisRespiratory MusclesRespiratory Function TestsBioassays and Physiological AnalysisMuscle relaxationInhalationGenetic DiseasesExhalationParasternal lineCardiologyFemaleAnatomyMuscle ElectrophysiologyResearch ArticleAdult; Anthropometry; Electromyography; Exhalation; Female; Humans; Inhalation; Male; Muscle Relaxation; Muscle Strength; Myotonic Dystrophy; Pressure; ROC Curve; Respiratory Function Tests; Respiratory Muscles; Sample Size; Time FactorsAdultmedicine.medical_specialtyRespiratory physiologyResearch and Analysis Methods03 medical and health sciencesInternal medicineRespiratory musclesPressureRespiratory muscleHumansRespiratory PhysiologyMuscle StrengthClinical GeneticsElectromyographic activityElectromyographybusiness.industryElectrophysiological Techniqueslcsh:RBiology and Life SciencesExhalationSkeletal MusclesROC Curve030228 respiratory systemSample Sizelcsh:Qbusiness030217 neurology & neurosurgery

description

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.

yearjournalcountryeditionlanguage
2017-06-08PLOS ONE
https://doi.org/10.1371/journal.pone.0177318