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. 2024 Aug;25(5-6):475-485.
doi: 10.1080/21678421.2024.2322545. Epub 2024 Mar 11.

Dyspnea (breathlessness) in amyotrophic lateral sclerosis/motor neuron disease: prevalence, progression, severity, and correlates

Carolyn A Young  1   2 , Amina Chaouch  3 , Christopher J Mcdermott  4 , Ammar Al-Chalabi  5   6 , Suresh K Chhetri  7 , Kevin Talbot  8 , Timothy Harrower  9 , Richard W Orrell  10 , Joe Annadale  11 , C Oliver Hanemann  12 , Antonio Scalfari  13 , Alan Tennant  14 , Roger Mills  1   2 ; TONIC-ALS STUDY GROUP
Affiliations

Dyspnea (breathlessness) in amyotrophic lateral sclerosis/motor neuron disease: prevalence, progression, severity, and correlates

Carolyn A Young et al. Amyotroph Lateral Scler Frontotemporal Degener. 2024 Aug.

Abstract

Objective: Dyspnea, or breathlessness, is an important symptom in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). We examined the measurement properties of the Dyspnea-12.

Methods: Rasch analysis enabled conversion of raw Dyspnea-12 scores to interval level metric equivalents. Converted data were used to perform trajectory modeling; those following different trajectories were compared for demographic, clinical, symptom, and functioning characteristics. Logistic regression examined differences between distinct trajectories.

Results: In 1022 people, at baseline, mean metric Dyspnea-12 was 7.6 (SD 9.3). 49.8% had dyspnea, severe in 12.6%. Trajectory analysis over 28 months revealed three breathlessness trajectories: group 1 reported none at baseline/follow-up (42.7%); group 2 significantly increased over time (9.4%); group 3 had a much higher level at baseline which rose over follow-up (47.9%). Group 3 had worse outcomes on all symptoms, functioning and quality of life; compared to group 1, their odds of: respiratory onset sixfold greater; King's stage ≥3 2.9 greater; increased odds of being bothered by choking, head drop, fasciculations, and muscle cramps; fatigue and anxiety also elevated (p < .01).

Conclusion: Dyspnea is a cardinal symptom in ALS/MND and can be quickly measured using the Dyspnea-12. Raw scores can easily be converted to interval level measurement, for valid change scores and trajectory modeling. Dyspnea trajectories reveal different patterns, showing that clinical services must provide monitoring which is customized to individual patient need. Almost half of this large population had worsening dyspnea, confirming the importance of respiratory monitoring and interventions being integrated into routine ALS care.

Keywords: Dyspnea; Rasch; breathlessness; measure; trajectories of outcome in neurological conditions-ALS.

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Conflict of interest statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
Dyspnea-12 item threshold map ordered by difficulty.
Figure 2.
Figure 2.
Dyspnea-12 trajectories. Fine dashed lines indicate 95% confidence limits.
Figure 3.
Figure 3.
Dyspnea-12 trajectories in inception group (for whom duration since diagnosis ≤ 6 months). Fine dashed lines indicate 95% confidence limits.
See this image and copyright information in PMC

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    Young CA, Chaouch A, Mcdermott CJ, Al-Chalabi A, Chhetri SK, Bidder C, Edmonds E, Ellis C, Annadale J, Wilde L, Sharrack B, Malaspina A, Leach O, Mills R, Tennant A. Young CA, et al. Amyotroph Lateral Scler Frontotemporal Degener. 2025 May;26(3-4):192-202. doi: 10.1080/21678421.2024.2435969. Epub 2025 Jan 3. Amyotroph Lateral Scler Frontotemporal Degener. 2025. PMID: 39749679 Free PMC article.

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