Assessing Prevalence and Characteristics of Oro-bulbar Involvement in Children and Adults with SMA Type 2 and 3 Using a Multimodal Approach
Articolo
Data di Pubblicazione:
2023
Citazione:
Assessing Prevalence and Characteristics of Oro-bulbar Involvement in Children and Adults with SMA Type 2 and 3 Using a Multimodal Approach / F. Trucco, F. Salmin, A. Lizio, G. Coratti, E. Albamonte, M.C. Frisoni, L. Mauro, E. Carraro, G. Palazzo, J. Lops, C. Cattaneo, S. Pozzi, J. Casiraghi, A. Di Bari, B. Berti, G. Stanca, M. Ricci, M. Pane, C. Heatwole, N. Dilek, E. Mercuri, V.A. Sansone. - In: DYSPHAGIA. - ISSN 0179-051X. - 38:6(2023 Dec), pp. 1568-1580. [10.1007/s00455-023-10584-z]
Abstract:
Bulbar and jaw muscles are impaired in patients with Spinal Muscular Atrophy (SMA) but the assessment of their severity and progression are limited by the lack of age-appropriate and disease-specific measures. We investigated mastication and swallowing in children and adults with SMA, sitters and walkers. In a 2-year multicentre cross-sectional prospective study, lip and tongue strength (Iowa Oral Performance Instrument), chewing and swallowing (Test of Masticating and Swallowing Solids), active mouth opening (aMMO) were compared to age-appropriate normative data. The perceived burden of oro-bulbar involvement (SMA-Health Index) was recorded. 78 patients were included, 45 children (median age 7.4 years),22 adults (median age 26.8 years) on nusinersen and 11 untreated (median age 32.7 years). Forty-three percent children had reduced mouth opening, 50% had prolonged total time to eat. These issues were more prominent in sitters than in walkers (p = 0.019, p = 0.014). Sixty-six percent needed increased swallows for bolus clearance. Nusinersen treated adults had median aMMO, tongue strength and total time at TOMASS values within normal range (z score: -1.40, -1.22, -1.32, respectively) whereas untreated adults had reduced aMMO (z score: -2.68) and tongue strength (z score: -2.20). Only a minority of children (2/17) and treated adults (5/21) reported burden in swallowing or mastication compared to all untreated adults (5/5). After 16 months, mastication and swallowing were stable in treated children and adults, whether sitters or walkers. The reported multimodal approach to assess oro-bulbar functions demonstrate that swallowing and mastication are impaired in SMA despite patients' perception. These results suggest a trend towards stabilization of oro-bulbar function in patients on long-term treatment with nusinersen.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Mastication; Nusinersen; Oro-bulbar involvement; Spinal muscular atrophy; Swallowing
Elenco autori:
F. Trucco, F. Salmin, A. Lizio, G. Coratti, E. Albamonte, M.C. Frisoni, L. Mauro, E. Carraro, G. Palazzo, J. Lops, C. Cattaneo, S. Pozzi, J. Casiraghi, A. Di Bari, B. Berti, G. Stanca, M. Ricci, M. Pane, C. Heatwole, N. Dilek, E. Mercuri, V.A. Sansone
Link alla scheda completa: