Diaphragm ultrasonography in amyotrophic lateral sclerosis: a diagnostic tool to assess ventilatory dysfunction and disease severity
- 109 Downloads
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.
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.
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).
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.
KeywordsNeuromuscular ultrasonography (NMUS) Diaphragm assessment Respiratory function Amyotrophic lateral sclerosis (ALS) Motor neuron disease (MND) Prognosis
All the authors have contributed to the conception and design of the study, analysis, and interpretation of data and drafting of the manuscript.
Compliance with ethical standards
Ethical publication statement
We confirm that we have read the journal’s position on issues involved in ethical publication and affirm that this study is consistent with those guidelines.
Disclosure of conflicts of interest
The authors declare that they have no conflict of interest.
- 10.Geevasinga N, Loy CT, Menon P, de Carvalho M, Swash M, Schrooten M, Van Damme P, Gawel M, Sonoo M, Higashihara M, Noto Y, Kuwabara S, Kiernan MC, Macaskill P, Vucic S (2016) Awaji criteria improves the diagnostic sensitivity in amyotrophic lateral sclerosis: a systematic review using individual patient data. Clin Neurophysiol 127(7):2684–2691CrossRefGoogle Scholar
- 16.Walker FO, Cartwright MS, Alter KE, Visser LH, Hobson-Webb LD, Padua L, Strakowski JA, Preston DC, Boon AJ, Axer H, van Alfen N, Tawfik EA, Wilder-Smith E, Yoon JS, Kim BJ, Breiner A, Bland JDP, Grimm A, Zaidman CM (2018) Indications for neuromuscular ultrasound: expert opinion and review of the literature. Clin Neurophysiol S1388–2457(18):31234–31233Google Scholar
- 24.Janssens AI, Ruytings M, Al-Chalabi A, Chio A, Hardiman O, Mcdermott CJ, Meyer T, Mora G, Van Damme P, Van Den Berg LH, Vanhaecht K, Winkler AS, Sermeus W, ALS-CARE Consortium (2016) A mapping review of international guidance on the management and care of amyotrophic lateral sclerosis (ALS). Amyotroph Lateral Scler Frontotemporal Degener 17(5–6):325–336CrossRefGoogle Scholar
- 25.Gil J, Funalot B, Verschueren A, Danel-Brunaud V, Camu W, Vandenberghe N, Desnuelle C, Guy N, Camdessanche JP, Cintas P, Carluer L, Pittion S, Nicolas G, Corcia P, Fleury MC, Maugras C, Besson G, Le Masson G, Couratier P (2008) Causes of death amongst French patients with amyotrophic lateral sclerosis: a prospective study. Eur J Neurol 15(11):1245–1251CrossRefGoogle Scholar
- 31.Fregonezi G, Araújo PR, Macêdo TL, Dourado Junior ME, Resqueti VR, Andrade Ade F (2013) Monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of pulmonary function evaluation in amyotrophic lateral sclerosis. Arq Neuropsiquiatr 71(3):146–152CrossRefGoogle Scholar