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. 2022 Dec;32(11-12):879-885.
doi: 10.1016/j.nmd.2022.11.004. Epub 2022 Nov 17.

Long term follow-up of scoliosis progression in type II SMA patients

Giorgia Coratti  1 , Maria Carmela Pera  1 , Adele D'Amico  2 , Claudio Bruno  3 , Francesca Bovis  4 , Consolato Gullì  5 , Noemi Brolatti  3 , Marina Pedemonte  3 , Massimo Apicella  6 , Laura Antonaci  1 , Martina Ricci  1 , Anna Capasso  1 , Gianpaolo Cicala  1 , Costanza Cutrona  6 , Roberto de Sanctis  7 , Sara Carnicella  7 , Nicola Forcina  7 , Michela Catteruccia  2 , Maria Beatrice Damasio  8 , Luca Labianca  9 , Antonio Leone  5 , Enrico Bertini  2 , Marika Pane  1 , Eugenio Mercuri  10
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Long term follow-up of scoliosis progression in type II SMA patients

Giorgia Coratti et al. Neuromuscul Disord. 2022 Dec.

Abstract

The aim of this study is to retrospectively assess onset and progression of scoliosis in type II SMA patients not treated with the approved disease modifying treatments. Scoliosis was evaluated by measuring the scoliosis angle on X-ray obtained in the anteroposterior view in sitting position (Cobb's angle method). Eighty-four patients had at least one assessment of scoliosis angle (287 assessments). There was a positive correlation between age and scoliosis angles (p<0.001) with a progressive increase of scoliosis with age. When subdividing the population by HFMSE score (<10; 11-22;> 22), there was a progressive increase in scoliosis angles with decreasing HFMSE scores. The difference between HFMSE categories was significant (p<0.001). Fifty-four patients had at least two assessments at 6-month distance and were retained for the longitudinal analysis. Using a mixed model, age, functional status and scoliosis angle at baseline were predictive on scoliosis progression. The mean annual rate of increase of scoliosis angle was 5.63 (95%CI: 4.74-6.52). Our results confirm the progression of scoliosis in untreated type II SMA providing details of the progression in relation to different variables. With different therapeutical options being available in many countries, our findings will provide reference data for establishing possible differences in the trajectories of progression with treated type II individuals.

Keywords: Cobb's angle; Natural history; Neuromuscular disorders; Scoliosis; Spinal muscular atrophy; Trajectories.

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Conflict of interest statement

Declaration of Competing Interest Giorgia Coratti reports personal fees from BIOGEN S.R.L. ITALIA, ROCHE, GENESIS PHARMA, AVEXIS, Biologix, outside the submitted work; Roberto de Sanctis reports personal fees from BIOGEN S.R.L. ITALIA and AVEXIS outside the submitted work; Maria C Pera reports personal fees from ROCHE outside the submitted work; Marika Pane reports personal fees from BIOGEN S.R.L., PTC, AVEXIS, SAREPTA, outside the submitted work; Adele D'Amico reports personal fees from BIOGEN S.R.L., SAREPTA, NOVARTIS, outside the submitted work; Enrico Bertini reports personal fees from BIOGEN S.R.L., AVEXIS, ROCHE, outside the submitted work; Claudio Bruno reports personal fees from Biogen, from Avexis, from Roche, outside the submitted work; Eugenio Mercuri reports personal fees from AVEXIS, ROCHE, BIOGEN S.R.L., PTC THERAPEUTICS, SAREPTA, SANTHERA, outside the submitted work; Francesca Bovis, Consolato Gullì, Noemi Brolatti, Marina Pedemonte, Massimo Apicella, Laura Antonaci, Martina Ricci, Anna Capasso, Gianpaolo Cicala, Costanza Cutrona, Sara Carnicella, Nicola Forcina, Michela Catteruccia, Maria Beatrice Damasio, Luca Labianca, Antonio Leone have nothing to disclose.

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