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Unstable control of breathing can lead to ineffective noninvasive ventilation in amyotrophic lateral sclerosis

Jesús Sancho, Enric Burés, Santos Ferrer, Ana Ferrando, Pilar Bañuls, Emilio Servera
ERJ Open Research 2019 5: 00099-2019; DOI: 10.1183/23120541.00099-2019
Jesús Sancho
1Respiratory Care Unit, Respiratory Medicine Dept, Hospital Clínico Universitario, Valencia, Spain
2Research Group for Respiratory Problems in Neuromuscular Diseases, Fundación para la Investigación HCUV-INCLIVA, Valencia, Spain
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  • For correspondence: jesus.sancho@uv.es
Enric Burés
1Respiratory Care Unit, Respiratory Medicine Dept, Hospital Clínico Universitario, Valencia, Spain
2Research Group for Respiratory Problems in Neuromuscular Diseases, Fundación para la Investigación HCUV-INCLIVA, Valencia, Spain
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Santos Ferrer
1Respiratory Care Unit, Respiratory Medicine Dept, Hospital Clínico Universitario, Valencia, Spain
2Research Group for Respiratory Problems in Neuromuscular Diseases, Fundación para la Investigación HCUV-INCLIVA, Valencia, Spain
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Ana Ferrando
3Respiratory Medicine Dept, Hospital Clínico Universitario, Valencia, Spain
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Pilar Bañuls
1Respiratory Care Unit, Respiratory Medicine Dept, Hospital Clínico Universitario, Valencia, Spain
2Research Group for Respiratory Problems in Neuromuscular Diseases, Fundación para la Investigación HCUV-INCLIVA, Valencia, Spain
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Emilio Servera
1Respiratory Care Unit, Respiratory Medicine Dept, Hospital Clínico Universitario, Valencia, Spain
2Research Group for Respiratory Problems in Neuromuscular Diseases, Fundación para la Investigación HCUV-INCLIVA, Valencia, Spain
4Physical Medicine Dept, Universitat de Valencia, Valencia, Spain
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  • FIGURE 1
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    FIGURE 1

    Polysomnography recording of an obstructive event in the upper airway with decreased central drive in a patient with amyotrophic lateral sclerosis using noninvasive ventilation in volume-preset mode.

  • FIGURE 2
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    FIGURE 2

    Relationship between minute ventilation (V′E) and end-tidal carbon dioxide tension (PETCO2) representing controller gain (CG) in two amyotrophic lateral sclerosis patients, one with obstruction with decreased central drive index (ODCDI) <5 h−1 and another with ODCDI >5 h−1.

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  • TABLE 1

    Demographic and pulmonary function in the total population and the groups with obstruction with decreased central drive index (ODCDI) <5 and >5 h−1

    Total populationODCDI <5 h−1ODCDI >5 h−1p-value
    Subjects30273
    Sex0.804
     Male18162
     Female12111
    Age years67.06±12.0867.18±12.3666.00±11.260.875
    BMI kg·m−226.19±4.5226.51±4.7023.69±0.810.319
    ALS onset0.051
     Spinal21210
     Bulbar963
    Time from ALS onset months38.53±28.3439.24±29.7132.66±13.860.712
    Time from NIV onset months10.96±16.9311.55±17.665.66±7.230.577
    ALSFRS-R22.13±8.6921.55±8.4527.33±10.960.282
    NBS19.56±12.9320.44±12.9111.66±12.420.272
    FVC L1.24±0.681.22±0.671.42±0.830.638
    FVC % pred41.49±21.6040.58±20.5949.66±33.850.500
    PImax cmH2O−27.82±17.50−27.60±17.82−29.66±17.950.851
    PEmax cmH2O46.67±36.9342.16±37.5442.00±38.350.835
    SNIP cmH2O−24.67±16.90−24.88±17.74−23.00±8.540.860
    MIC L1.54±0.791.53±0.801.61±0.920.871
    PCF L·s−12.68±1.402.65±1.392.97±1.750.719
    PCFMIC L·s−13.21±1.573.22±1.583.12±1.800.917
    PCFMI-E L·s−13.02±0.933.05±0.982.78±0.220.649

    BMI: body mass index; ALS: amyotrophic lateral sclerosis; NIV: noninvasive ventilation; ALSFRS-R: Revised Amyotrophic Lateral Sclerosis Functional Rating Scale; NBS: Norris scale bulbar subscore; FVC: forced vital capacity; PImax: maximum inspiratory pressure; PEmax: maximum expiratory pressure; SNIP: sniff nasal inspiratory pressure; MIC: maximum insufflation capacity; PCF: peak cough flow; PCFMIC: manually assisted PCF; PCFMI-E: mechanically assisted PCF.

    • TABLE 2

      Polysomnography while using noninvasive ventilation and gas exchange parameters in the groups with obstruction with decreased central drive index (ODCDI) <5 and >5 h−1

      ODCDI <5 h−1ODCDI >5 h−1p-value
      Subjects273
      TST min339.85±76.42364.80±95.610.610
      Sleep latency min18.13±15.6718.26±21.010.989
      Sleep efficiency index %68.83±12.1474.16±7.300.469
      Arousal/awakening index h−119.31±18.2728.50±27.780.447
      Sleep stage
       N1 %24.17±12.2621.40±13.230.719
       N2 %46.95±13.4249.03±3.090.795
       N3 %23.17±14.1428.50±16.290.552
       REM %5.67±5.511.03±0.110.001
      TST90 %3.77±7.297.70±7.630.386
      ODI h−16.79±12.5428.30±20.030.012
      Minimum SpO2 %84.70±9.6681.00±7.210.528
      Mean SpO2 %94.29±1.8993.33±1.150.401
      ODCDI h−10.88±0.9912.99±5.770.028
      pH7.42±0.027.42±0.020.865
      PaO2 mmHg79.92±8.5769.66±10.010.062
      PaCO2 mmHg42.37±4.2748.66±7.570.032
      HCO3 mmol·L−127.48±2.2929.40±4.800.562

      TST: total sleep time; REM: rapid eye movement; TST90: time spent with arterial oxygen saturation <90%; ODI: oxygen desaturation index; SpO2: arterial oxygen saturation measured by pulse oximetry; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; HCO3: bicarbonate.

      • TABLE 3

        Associations with the obstruction with decreased central drive index

        r-valuep-value
        Age−0.090.609
        BMI−0.220.269
        Time from ALS onset−0.080.673
        Time from NIV onset−0.150.429
        Daily hours of use of NIV−0.180.323
        V′E with NIV−0.240.205
        ALSFRS-R0.020.883
        NBS−0.370.044
        FVC0.010.958
        FVC % pred−0.020.919
        PImax−0.100.588
        PEmax0.100.592
        SNIP0.130.948
        MIC−0.050.779
        PCF−0.030.857
        PCFMIC−0.130.504
        PCFMI-E−0.100.587
        Apnoeic threshold−0.020.901
        CO2R−0.350.037
        Controller gain0.590.001
        Plant gain−0.230.222

        BMI: body mass index; ALS: amyotrophic lateral sclerosis; NIV: noninvasive ventilation; V′E: minute ventilation; ALSFRS-R: Revised Amyotrophic Lateral Sclerosis Functional Rating Scale; NBS: Norris scale bulbar subscore; FVC: forced vital capacity; PImax: maximum inspiratory pressure; PEmax: maximum expiratory pressure; SNIP: sniff nasal inspiratory pressure; MIC: maximum insufflation capacity; PCF: peak cough flow; PCFMIC: manually assisted PCF; PCFMI-E: mechanically assisted PCF; CO2R: carbon dioxide reserve.

        Supplementary Materials

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          Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.

          Supplementary material 00099-2019.supp

          Video 1 00099-2019.video1

          Video 2 00099-2019.video2

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        Unstable control of breathing can lead to ineffective noninvasive ventilation in amyotrophic lateral sclerosis
        Jesús Sancho, Enric Burés, Santos Ferrer, Ana Ferrando, Pilar Bañuls, Emilio Servera
        ERJ Open Research Jul 2019, 5 (3) 00099-2019; DOI: 10.1183/23120541.00099-2019

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        Unstable control of breathing can lead to ineffective noninvasive ventilation in amyotrophic lateral sclerosis
        Jesús Sancho, Enric Burés, Santos Ferrer, Ana Ferrando, Pilar Bañuls, Emilio Servera
        ERJ Open Research Jul 2019, 5 (3) 00099-2019; DOI: 10.1183/23120541.00099-2019
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