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Abstract
Objectives Disease-modifying agents (DMAs) for the treatment of spinal muscular atrophy (SMA) have evolved the SMA phenotype with improved survival. Ongoing oropharyngeal dysphagia and respiratory complications are reported. The extent of dysphagia and respiratory morbidity in this population, since DMAs' introduction, has not been well described.
Methods A whole-population study involved all children with treated SMA types 1–3 in our facility. Videofluoroscopic swallow studies (type 1 alone), chest CT scans, and clinical data were collected.
Results Thirty-six children were included (n = 9 type 1, n = 14 type 2, and n = 13 type 3; age range 0.3–15.4 years). Abnormal swallowing characteristics were demonstrated in all children with type 1 (n = 8; 100%). Bronchiectasis was found on chest CT: 3 of 9 (33.3%), 2 of 14 (14.3%), and 2 of 13 (15.4%) of type 1, 2, and 3, respectively. Atelectasis, mucus plugging, bronchial wall thickening, and parenchymal changes were common.
Discussion Swallow impairments were universal in children with type 1. Bronchiectasis was common in all pediatric SMA types, with a prevalence of 1 in 5. Routine monitoring and management of dysphagia/recurrent respiratory infection should be implemented for improvement in lung health.
Footnotes
Submitted and externally peer reviewed. The handling editor was Associate Editor Courtney Wusthoff, MS, MS.
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received July 12, 2022.
- Accepted in final form December 2, 2022.
- © 2023 American Academy of Neurology
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