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Diaphragm ultrasonography in amyotrophic lateral sclerosis: a diagnostic tool to assess ventilatory dysfunction and disease severity

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Abstract

Background

Respiratory failure represents an unavoidable step in patients with amyotrophic lateral sclerosis (ALS) and other motor neuron diseases (MND). The development of diaphragm ultrasonography (DUS) provides an alternative useful and risk-free tool to supply clinical, functional, and neurophysiological assessment of respiratory muscle weakness. Our aim was to evaluate if sonographic changes (thickness and echogenicity in the costal portion of the diaphragm, at rest and during respiratory movements) may be used in ALS patients to assess disease severity over time, to rule out any risk or discomfort due to traditional neurophysiological investigations.

Methods

Twenty ALS patients (mean age, 64.6 ± 10.5 years) were enrolled and data were compared with age-matched healthy volunteers; DUS data were correlated with respiratory function and disease severity scale. Examinations were performed using Telemed Echo-wave II or Esaote MyLabGamma devices in conventional B-Mode.

Results

Mean resting thickness was reduced in all cases; changes in thickness during inspiration and expiration were also reduced (p < 0.0001) and lost in severe cases (n = 3). In bulbar-onset disease, respiratory scores were strictly correlated with the difference in diaphragm thickness between full inspiration—and expiration—as well as on the diaphragm thickness in expiration (p < 0.001).

Conclusions

DUS represents a simple, painless, and risk-free tool; moreover, it provides useful functional and structural insights to the understanding of diaphragm function and the degree of respiratory failure in ALS.

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Authors and Affiliations

  1. Department of Clinical and Experimental Medicine, Neurology - Neurophysiopathology Unit, Pisa University Medical School, Cisanello Hospital, Via Paradisa, n. 2, 56124, Pisa, Italy

    Ferdinando Sartucci, Michela Santin & Tommaso Bocci

  2. Integrated Care Department of Medical Specialties, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy

    Ferdinando Sartucci & Michela Santin

  3. CNR Neuroscience Institute, Pisa, Italy

    Ferdinando Sartucci

  4. Acquired Severe Brain Injuries Department Section, Cisanello University Hospital, Pisa, Italy

    Alessio Pelagatti, Cristina Dolciotti & Paolo Bongioanni

  5. Neurorehabilitation Unit, University of Pisa, Pisa, Italy

    Alessio Pelagatti, Cristina Dolciotti & Paolo Bongioanni

  6. “Aldo Ravelli” Center for Neurotechnology and Experimental Brain Therapeutics, Department of Health Sciences, University of Milan & ASST Santi Paolo e Carlo, Milan, Italy

    Tommaso Bocci

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  1. Ferdinando Sartucci
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  2. Alessio Pelagatti
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  4. Tommaso Bocci
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  5. Cristina Dolciotti
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  6. Paolo Bongioanni
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Contributions

All the authors have contributed to the conception and design of the study, analysis, and interpretation of data and drafting of the manuscript.

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Correspondence to Ferdinando Sartucci.

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Sartucci, F., Pelagatti, A., Santin, M. et al. Diaphragm ultrasonography in amyotrophic lateral sclerosis: a diagnostic tool to assess ventilatory dysfunction and disease severity. Neurol Sci 40, 2065–2071 (2019). https://doi.org/10.1007/s10072-019-03938-9

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  • DOI: https://doi.org/10.1007/s10072-019-03938-9

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