MRI patterns of muscle involvement in type 2 and 3 spinal muscular atrophy patients

Tommaso Tartaglione, Cesare Colosimo, Eugenio Maria Mercuri, Tommaso Verdolotti, Maria Carmela Pera, Laura Antonaci, Luana Ficociello, Giorgio Tasca, Anna Pichiecchio, Claudia Cinnante, Valeria Sansone, Luca Maria Sconfienza, Angela Berardinelli, Matteo Garibaldi, Giovanni Antonini, Emilio Albamonte, Chiara Begliuomini, Lorenzo Maggi, Alessandra Govoni, Giacomo Comi

Risultato della ricerca: Contributo in rivistaArticolo in rivista

8 Citazioni (Scopus)

Abstract

Only few studies have reported muscle involvement in spinal muscular atrophy using muscle MRI but this has not been systematically investigated in a large cohort of both pediatric and adult patients with type 2 and type 3 spinal muscular atrophy. The aim of the present study was to define possible patterns of muscle involvement on MRI, assessing both fatty replacement and muscle atrophy, in a cohort of type 2 and type 3 spinal muscular atrophy children and adults (age range 2–45 years), including both ambulant and non-ambulant patients. Muscle MRI protocol consisted in T1-weighted sequences acquired on axial plane covering the pelvis, the thigh, and the leg with contiguous slices. Each muscle was examined through its whole extension using a grading system that allows a semiquantitative evaluation of fatty infiltration. Thigh muscles were also grouped in anterior, posterior, and medial compartment for classification of global atrophy. The results showed a large variability in both type 2 and type 3 spinal muscular atrophy, with a various degree of proximal to distal gradient. Some muscles, such us the adductor longus and gracilis were always selectively spared. In all patients, the involvement was a combination of muscle atrophy and muscle infiltration. The variability observed may help to better understand both natural history and response to new treatments.
Lingua originaleEnglish
pagine (da-a)898-912
Numero di pagine15
RivistaJournal of Neurology
Volume267
DOI
Stato di pubblicazionePubblicato - 2020

Keywords

  • Fatty infiltration
  • Magnetic Resonance Imaging
  • Muscle atrophy
  • Spinal muscular atrophy

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