Functional levels and MRI patterns of muscle involvement in upper limbs in Duchenne muscular dystrophy

Claudia Brogna, Lara Cristiano, Tommaso Tartaglione, Tommaso Verdolotti, Lavinia Fanelli, Luana Ficociello, Giorgio Tasca, Roberta Battini, Giorgia Coratti, Nicola Forcina, Roberto De Santis, Giulia Norcia, Sara Carnicella, Cesare Colosimo, Pierre Carlier, Marika Pane, Eugenio Mercuri

Research output: Contribution to journalArticle

Abstract

The aim of the study was to evaluate the spectrum of upper limb functional activities and imaging finding in a cohort of patients affected by Duchenne muscular dystrophy. Thirty-one patients of age between 5 and 29 years were included in the study (17 ambulant and 14 non-ambulant). They were all assessed using the Performance of Upper Limb (PUL) test and muscle MRI of shoulder, arm and forearm in order to establish if the functional scores obtained at shoulder, mid and distal level related to specific patterns of involvement in each upper limb segment on muscle MRI. At shoulder level, latissimus dorsi, serratus anterior, infraspinatus and subscapularis were always involved, even in patients with full functional scores at shoulder level. Diffuse and severe involvement of all muscles was found in the patients with a PUL shoulder functional score of 5. At arm level biceps brachii, brachialis and triceps were generally concordantly involved or spared. Some degree of involvement could already be detected in patients with reduced scores on the PUL mid domain. They were generally severely involved in patients with functional scores less than 6 at mid-level. At distal level supinator and pronator muscles were often involved, followed by brachioradialis and, less frequently, by the muscles of the flexor compartment. The extensor muscles were generally completely spared. A diffuse and severe involvement was found only in patients who had very low scores (8 or below) on the PUL distal domain. The integrated use of functional scales and imaging allowed to establish patterns of involvement at each level, and the functional scores that were more frequently associated with diffuse and severe involvement.

Original languageEnglish
Article numbere0199222
JournalPLoS One
Volume13
Issue number6
DOIs
Publication statusPublished - Jun 1 2018

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Fingerprint Dive into the research topics of 'Functional levels and MRI patterns of muscle involvement in upper limbs in Duchenne muscular dystrophy'. Together they form a unique fingerprint.

  • Cite this

    Brogna, C., Cristiano, L., Tartaglione, T., Verdolotti, T., Fanelli, L., Ficociello, L., Tasca, G., Battini, R., Coratti, G., Forcina, N., De Santis, R., Norcia, G., Carnicella, S., Colosimo, C., Carlier, P., Pane, M., & Mercuri, E. (2018). Functional levels and MRI patterns of muscle involvement in upper limbs in Duchenne muscular dystrophy. PLoS One, 13(6), [e0199222]. https://doi.org/10.1371/journal.pone.0199222