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Peer-reviewedCC BY-NC 4.0Multidisciplinary Respiratory Medicine

Noninvasive ventilation in patients with amyotrophic lateral sclerosis: Is home sleep apnea testing a useful tool for initiating noninvasive ventilation?

  1. 1Research Center of Sleep Disorders, Neurology Unit, AOU Cagliari, Hospital D. Casula Monserrato, Cagliari, Italy
  2. 2ALS Center, Neurology Unit, AOU Cagliari, Hospital D. Casula Monserrato, Cagliari, Italy
  3. 3Department of Medical Science and Public Health, University of Cagliari, Cagliari, Italy

Abstract

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by motor neuron degeneration and eventual respiratory failure, often first manifesting during sleep. Timely detection of nocturnal respiratory impairment is critical for initiating noninvasive ventilation (NIV), one of the few therapies proven to improve survival. Although polysomnography (PSG) is the reference standard, it is not always readily available in clinical practice.

Case presentation: We describe a 62-year-old man with spinal-onset ALS who underwent home sleep apnea testing (HSAT) for respiratory assessment. Although standard metrics were within normal limits, careful manual analysis of respiratory signals revealed diaphragmatic dysfunction through position-dependent desaturation and paradoxical breathing. NIV was initiated, resulting in improvements in sleep quality and daytime functioning.

Conclusions: This case illustrates the value of HSAT as a pragmatic and accessible tool for early detection of respiratory involvement in ALS, particularly when combined with detailed waveform inspection in setting where PSG is unavailable. Additionally, it emphasizes the importance of individualized NIV titration and interface optimization to ensure adherence and therapeutic benefit.

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BibTeX
@article{sanna2026noninvasive,
  title = {{Noninvasive ventilation in patients with amyotrophic lateral sclerosis: Is home sleep apnea testing a useful tool for initiating noninvasive ventilation?}},
  author = {Chiara Sanna and Mauro Sanna and Giuseppe Borghero and Stefania Redolfi},
  journal = {Multidisciplinary Respiratory Medicine},
  year = {2026},
  month = sep,
  doi = {10.5826/mrm.2026.1088},
  url = {https://doi.org/10.5826/mrm.2026.1088},
  note = {CC BY-NC 4.0},
}
RIS
TY  - JOUR
TI  - Noninvasive ventilation in patients with amyotrophic lateral sclerosis: Is home sleep apnea testing a useful tool for initiating noninvasive ventilation?
AU  - Chiara Sanna
AU  - Mauro Sanna
AU  - Giuseppe Borghero
AU  - Stefania Redolfi
PY  - 2026
DA  - 2026/09/16
JO  - Multidisciplinary Respiratory Medicine
DO  - 10.5826/mrm.2026.1088
UR  - https://doi.org/10.5826/mrm.2026.1088
AB  - Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by motor neuron degeneration and eventual respiratory failure, often first manifesting during sleep. Timely detection of nocturnal respiratory impairment is critical for initiating noninvasive ventilation (NIV), one of the few therapies proven to improve survival. Although polysomnography (PSG) is the reference standard, it is not always readily available in clinical practice.  Case presentation: We describe a 62-year-old man with spinal-onset ALS who underwent home sleep apnea testing (HSAT) for respiratory assessment. Although standard metrics were within normal limits, careful manual analysis of respiratory signals revealed diaphragmatic dysfunction through position-dependent desaturation and paradoxical breathing. NIV was initiated, resulting in improvements in sleep quality and daytime functioning.  Conclusions: This case illustrates the value of HSAT as a pragmatic and accessible tool for early detection of respiratory involvement in ALS, particularly when combined with detailed waveform inspection in setting where PSG is unavailable. Additionally, it emphasizes the importance of individualized NIV titration and interface optimization to ensure adherence and therapeutic benefit.
SN  - 1828-695X
SN  - 2049-6958
ER  -