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

  • Grazia Crescimanno
  • Marcello Romano
  • Rossella Spataro
  • Vincenzo La Bella
  • Oreste Marrone
Letter to the Editor
  • 25 Downloads

Introduction

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...

Notes

Acknowledgements

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.

References

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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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