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. 2024 Aug;23(4):1338-1347.
doi: 10.1007/s12311-023-01635-0. Epub 2023 Nov 30.

Sensorimotor Cough Dysfunction in Cerebellar Ataxias

Affiliations

Sensorimotor Cough Dysfunction in Cerebellar Ataxias

Emilie R Lowell et al. Cerebellum. 2024 Aug.

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.

Keywords: Cerebellar ataxias; Cough; Cough expired volume; Peak expiratory flow rate.

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Conflict of interest statement

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.

Figures

Fig. 1
Fig. 1
A cough airflow waveform depicts a cough epoch with three coughs (CrTot = 3). Coughs are indicated by a corresponding cough response marker (e.g., Cr1). Peak expiratory flow rate (PEFR) and cough expired volume (CEV) are indicated for the first cough in the epoch
Fig. 2
Fig. 2
Bar charts of urge-to-cough ratings by diagnosis (idiopathic late onset cerebellar ataxia (ILOCA) or spinocerebellar ataxia (SCA) subtype 1, 2, or 3). The urge-to-cough distribution of each group is in dark gray, superimposed over light gray representing the entire cohort
Fig. 3
Fig. 3
Graphs illustrate voluntary and reflex cough peak expiratory flow rate (PEFR) plotted against ataxia severity, as measured by the Scale for the Assessment and Rating of Ataxia (SARA). A line of best fit is depicted for voluntary cough, along with gray shading representing a 95% confidence interval
Fig. 4
Fig. 4
Graphs illustrate voluntary and reflex cough expired volume (CEV) plotted against ataxia severity, as measured by the Scale for the Assessment and Rating of Ataxia (SARA). A line of best fit is depicted for voluntary cough, along with gray shading representing a 95% confidence interval

References

    1. Paulson HL. The spinocerebellar ataxias. J Neuroophthalmol 2009;29(3):227–37. - PMC - PubMed
    1. Abdulmassih EM, da Teive S, Santos HAG. The evaluation of swallowing in patients with spinocerebellar ataxia and oropharyngeal dysphagia: a comparison study of videofluoroscopic and sonar doppler. Int Arch Otorhinolaryngol 2013;17(1):66–73. - PMC - PubMed
    1. Isono C, Hirano M, Sakamoto H, Ueno S, Kusunoki S, Nakamura Y. Differences in dysphagia between spinocerebellar ataxia type 3 and type 6. Dysphagia 2013;28(3):413–8. - PubMed
    1. Isono C, Hirano M, Sakamoto H, Ueno S, Kusunoki S, Nakamura Y. Progression of dysphagia in spinocerebellar ataxia type 6. Dysphagia 2017;32(3):420–6. - PubMed
    1. Jardim LB, Pereira ML, Silveira I, Ferro A, Sequeiros J, Giugliani R. Neurologic findings in machado-joseph Disease: relation with Disease duration, subtypes, and (cag)n. Arch Neurol 2001;58(6):899–904. - PubMed

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