Introduction: The effects of nusinersen in adults with SMA rely on neuromotor function scales and qualitative assessments. There are limited clinical or imaging data on muscle changes over time. Methods: Two adult SMA patients underwent clinical assessments including measures of upper and lower limb function with Revised Upper Limb Module (RULM) and Hammersmith Function Motor Scale Expanded (HFMSE); both patients were also studied with whole-body muscle MRI (T1-weighted and Diffusion Tensor Imaging/DTI sequences), at baseline and after 10 and 24 months from the beginning of treatment with nusinersen. Results: After two years of treatment, HFMSE and RULM scores were stable in both patients. DTI sequences revealed an increased number, length and organization of muscle fiber tracks, and Fractional Anisotropy (FA) values showed a significant reduction after 10 and 24 months from baseline, in their corresponding maps. Discussion: Muscle DTI imaging seems to play an interesting role to monitor treatment effects over time in adult SMA patients.
Muscle MRI in two SMA patients on nusinersen treatment : A two years follow-up / A. Barp, E. Carraro, E. Albamonte, F. Salmin, C. Lunetta, G.P. Comi, C. Messina, D. Albano, V. Chianca, L.M. Sconfienza, E.M. Mercuri, V. Sansone. - In: JOURNAL OF THE NEUROLOGICAL SCIENCES. - ISSN 0022-510X. - 417(2020 Oct 15), pp. 117067.1-117067.6.
|Titolo:||Muscle MRI in two SMA patients on nusinersen treatment : A two years follow-up|
|Parole Chiave:||Diffusion tensor imaging; Fractional anisotropy; Muscle MRI; Nusinersen; Spinal muscular atrophy; T1-weighted sequences|
|Settore Scientifico Disciplinare:||Settore MED/26 - Neurologia|
Settore MED/36 - Diagnostica per Immagini e Radioterapia
|Data di pubblicazione:||15-ott-2020|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1016/j.jns.2020.117067|
|Appare nelle tipologie:||01 - Articolo su periodico|