Muscle MRI in two SMA patients on nusinersen treatment: A two years follow-up

Andrea Barp, Elena Carraro, Emilio Albamonte, Francesca Salmin, Christian Lunetta, Giacomo Pietro Comi, Carmelo Messina, Domenico Albano, Vito Chianca, Luca Maria Sconfienza, Eugenio Maria Mercuri, Valeria Ada Sansone

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Article number117067
JournalJournal of the Neurological Sciences
Volume417
DOIs
Publication statusPublished - Oct 15 2020

Keywords

  • Diffusion tensor imaging
  • Fractional anisotropy
  • Muscle MRI
  • Nusinersen
  • Spinal muscular atrophy
  • T1-weighted sequences

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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