Muscle MRI in two SMA patients on nusinersen treatment: A two years follow-up
| العنوان: | Muscle MRI in two SMA patients on nusinersen treatment: A two years follow-up |
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| المؤلفون: | Domenico Albano, Andrea Barp, Eugenio Mercuri, Vito Chianca, Christian Lunetta, Francesca Salmin, Giacomo P. Comi, Emilio Albamonte, Elena Carraro, Luca Maria Sconfienza, Valeria A. Sansone, Carmelo Messina |
| المصدر: | Journal of the Neurological Sciences |
| بيانات النشر: | Elsevier B.V., 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Adult, medicine.medical_specialty, Oligonucleotides, DTI, Diffusion Tensor Imaging, Spinal Muscular Atrophies of Childhood, Muscle MRI, Article, MRI, Magnetic Resonance Imaging, 03 medical and health sciences, 0302 clinical medicine, Nusinersen, Fractional anisotropy, Medicine, Humans, SMA, Spinal Muscular Atrophy, 030212 general & internal medicine, Muscle fibre, SMN1, Survival of Motor Neuron 1 gene, HFMSE, Hammersmith Function Motor Scale Expanded, Muscle mri, RULM, Revised Upper Limb Module, business.industry, FA, Fractional Anisotropy, Muscles, Spinal muscular atrophy, SMA, medicine.disease, MRC, Medical Research Council, Magnetic Resonance Imaging, medicine.anatomical_structure, Diffusion Tensor Imaging, Neurology, T1-weighted sequences, Upper limb, Neurology (clinical), Radiology, business, 030217 neurology & neurosurgery, Diffusion MRI, Follow-Up Studies |
| الوصف: | 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. Highlights • Nusinersen treatment has created great expectations in older SMA patients having long-lasting muscular atrophy. • DTI is a very sensitive technique to identify small changes in muscle architecture. • DTI shows that nusinersen treatment may have a positive effect on size, length and organization of fiber tracts. |
| اللغة: | English |
| تدمد: | 1878-5883 0022-510X |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8a9ef3119d96237c614d54433936b0a0Test http://europepmc.org/articles/PMC7388822Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....8a9ef3119d96237c614d54433936b0a0 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 18785883 0022510X |
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