TY - JOUR
T1 - Muscle MRI in two SMA patients on nusinersen treatment
T2 - A two years follow-up
AU - Barp, Andrea
AU - Carraro, Elena
AU - Albamonte, Emilio
AU - Salmin, Francesca
AU - Lunetta, Christian
AU - Comi, Giacomo Pietro
AU - Messina, Carmelo
AU - Albano, Domenico
AU - Chianca, Vito
AU - Sconfienza, Luca Maria
AU - Mercuri, Eugenio Maria
AU - Sansone, Valeria Ada
PY - 2020/10/15
Y1 - 2020/10/15
N2 - 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.
AB - 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.
KW - Diffusion tensor imaging
KW - Fractional anisotropy
KW - Muscle MRI
KW - Nusinersen
KW - Spinal muscular atrophy
KW - T1-weighted sequences
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U2 - 10.1016/j.jns.2020.117067
DO - 10.1016/j.jns.2020.117067
M3 - Article
AN - SCOPUS:85088874642
VL - 417
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
M1 - 117067
ER -