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Sensorimotor Cough Dysfunction in Cerebellar Ataxias

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Abstract

Cerebellar ataxias are neurological conditions with a high prevalence of aspiration pneumonia and dysphagia. Recent research shows that sensorimotor cough dysfunction is associated with airway invasion and dysphagia in other neurological conditions and may increase the risk of pneumonia. Therefore, this study aimed to characterize sensorimotor cough function and its relationship with ataxia severity. Thirty-seven participants with cerebellar ataxia completed voluntary and/or reflex cough testing. Ataxia severity was assessed using the Scale for the Assessment and Rating of Ataxia (SARA). Linear multilevel models revealed voluntary cough peak expiratory flow rate (PEFR) estimates of 2.61 L/s and cough expired volume (CEV) estimates of 0.52 L. Reflex PEFR (1.82 L/s) and CEV (0.34 L) estimates were lower than voluntary PEFR and CEV estimates. Variability was higher for reflex PEFR (15.74% coefficient of variation [CoV]) than voluntary PEFR (12.13% CoV). 46% of participants generated at least two, two-cough responses following presentations of reflex cough stimuli. There was a small inverse relationship between ataxia severity and voluntary PEFR (β = -0.05, 95% CI: -0.09 – -0.01 L) and ataxia severity and voluntary CEV (β = -0.01, 95% CI: -0.02 – -0.004 L/s). Relationships between reflex cough motor outcomes (PEFR β = 0.03, 95% CI: -0.007–0.07 L/s; CEV β = 0.007, 95% CI: -0.004–0.02 L) and ataxia severity were not statistically robust. Results indicate that voluntary and reflex cough sensorimotor dysfunction is present in cerebellar ataxias and that increased severity of ataxia symptoms may impact voluntary cough function.

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Data Availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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Acknowledgements

The authors would like to thank our colleagues at the Laboratory for the Study of Upper Airway Dysfunction at Teachers College, Columbia University and the Department of Neurology at Columbia University Medical Center for their help with the recruitment of patients. We also thank our participants for their contribution to this research.

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Authors and Affiliations

  1. Laboratory for the Study of Upper Airway Dysfunction, Teachers College, Columbia University, 525 W 120th St, New York, NY, 10027, USA

    Emilie R. Lowell, James C. Borders, Avery E. Dakin, Jordanna S. Sevitz & Michelle S. Troche

  2. Department of Medicine, University of Otago Christchurch, Christchurch, New Zealand

    Sarah E. Perry & Michelle S. Troche

  3. New Zealand Brain Research Institute, Christchurch, New Zealand

    Sarah E. Perry

  4. University of Canterbury Rose Centre for Stroke Recovery & Research at St. George’s Medical Centre, Christchurch, New Zealand

    Sarah E. Perry

  5. Department of Neurology, Columbia University Medical Center, New York, NY, USA

    Sheng-Han Kuo

  6. The Initiative for Columbia Ataxia and Tremor, Columbia University, New York, NY, USA

    Sheng-Han Kuo

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  1. Emilie R. Lowell
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Contributions

(1) Research Project: (A) Conception, (B) Organization, (C) Execution. (2) Statistical Analysis: (A) Design, (B) Execution, (C) Review and Critique. (3) Manuscript: (A) Writing of the First Draft, (B) Review and Critique. E.R.L.: 1A, 1B, 1C, 2A, 2B, 2C, 3A, 3B. J.C.B: 1A, 1B, 1C, 2A, 2B, 2C, 3A, 3B. S.H.K.: 1A, 1B, 1C, 2C, 3A, 3C. S.E.P.: 1A, 1B, 1C, 2C, 3A, 3C. A.E.D.: 1A, 1B, 1C, 2C, 3A, 3C. J.S.S.: 1A, 1B, 1C, 2C, 3A, 3C. M.S.T.: 1A, 1B, 2A, 2C, 3A, 3B, 3C.

Corresponding author

Correspondence to Michelle S. Troche.

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Conflict of Interest

S.E.P. receives grant funding/salary for employment at the University of Otago/Neurological Foundation of New Zealand. S.H.K receives salary for employment at Columbia University, and serves on advisory boards for Praxis, Sage Therapeutics, Reata, and Biohaven. M.S.T. receives salary for employment at Teachers College, Columbia University, grant funding from NIH/NINDS, and royalties for consulting at MedBridge. M.S.T. also serves as a board member for the Dysphagia Research Society. No other authors have financial disclosures or conflicts of interest to report.

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The studies included in this manuscript were approved by the institutional review board of Teachers College at Columbia University (19–321, 15–430). We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.

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Lowell, E.R., Borders, J.C., Perry, S.E. et al. Sensorimotor Cough Dysfunction in Cerebellar Ataxias. Cerebellum (2023). https://doi.org/10.1007/s12311-023-01635-0

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