Neurological Sciences

, Volume 40, Issue 2, pp 421–422 | Cite as

Early and rapidly progressing respiratory failure in a patient with amyotrophic lateral sclerosis: when FVC% is misleading

  • Grazia CrescimannoEmail author
  • Marcello Romano
  • Rossella Spataro
  • Vincenzo La Bella
  • Oreste Marrone
Letter to the Editor


Respiratory failure is the leading cause of death in amyotrophic lateral sclerosis (ALS) [1]. The involvement of respiratory function is a negative predictor of survival, which can be in part overcome by a prompt initiation of non-invasive mechanical ventilation (NIV).

Seated forced vital capacity (FVC%) is a widely used measure to monitor respiratory function, and it is often taken as a surrogate outcome measure in clinical trials. However, it may be within a normal range in ALS patients with respiratory dysfunction [2].

Here we report on a case of an ALS patient with a rapidly evolving respiratory insufficiency, despite a rather normal FVC%.

Case presentation

The patient, a 67-year-old man with non-significant past medical history, was referred to our Neurology Unit with 7-month history of progressive impairment of neck muscles that caused dropped head. Symptom onset was subtle, and it was followed by camptocormia. The neurological examination documented the camptocormic...



The authors would like to thank Dr. G.Cangemi for his work in preparing this letter.

Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest.


  1. 1.
    Spataro R, Lo Re M, Piccoli T, Piccoli F, La Bella V (2010) Causes and place of death in Italian patients with amyotrophic lateral sclerosis. Acta Neurol Scand 122:217–223CrossRefGoogle Scholar
  2. 2.
    Atalaia A, De Carvalho M, Evangelista T, Pinto A (2007) Sleep characteristics of amyotrophic lateral sclerosis in patients with preserved diaphragmatic function. Amyotroph Lateral Scler 8:101–105CrossRefGoogle Scholar
  3. 3.
    Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sci 169:13–21CrossRefGoogle Scholar
  4. 4.
    Pinto S, de Carvalho M (2013) Dropped head presentation in ALS. Amyotroph Lateral Scler Frontotemporal Degener 14:235CrossRefGoogle Scholar
  5. 5.
    Tilanus TBM, Groothuis JT, TenBroek-Pastoor JMC, Feuth TB, Heijdra YF, Slenders JPL, Doorduin J, Van Engelen BG, Kampelmacher MJ, Raaphorst J (2017) The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis. Respir Res 18:144CrossRefGoogle Scholar
  6. 6.
    Polkey MI, Lyall RA, Yang K, Johnson E, Leigh PN, Moxham J (2017) Respiratory muscle strength as a predictive biomarker for survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med 195:86–95CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia S.r.l., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Istituto di Biomedicina e Immunologia MolecolareConsiglio Nazionale delle RicerchePalermoItaly
  2. 2.Centro regionale di riferimento perla prevenzione ed il trattamento delle malattie neuromuscolari genetiche rareOspedale Villa Sofia-CervelloPalermoItaly
  3. 3.Dipartimento di NeurologiaOspedale Villa Sofia-CervelloPalermoItaly
  4. 4.IRCCS Centro Neurolesi Bonino PulejoPalermoItaly
  5. 5.Centro di ricerca clinica sulla sclerosi laterale amiotrofica e Laboratorio di neurochimicaUniversità di PalermoPalermoItaly

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