Gain and loss of upper limb abilities in Duchenne muscular dystrophy patients: A 24-month study

Giorgia Coratti, Marika Pane, Claudia Brogna, Adele D'Amico, Elena Pegoraro, Luca Bello, Valeria A. Sansone, Emilio Albamonte, Elisabetta Ferraroli, Elena Stacy Mazzone, Lavinia Fanelli, Sonia Messina, Maria Sframeli, Michela Catteruccia, Gianpaolo Cicala, Anna Capasso, Martina Ricci, Silvia Frosini, Giacomo De Luca, Enrica RolleRoberto De Sanctis, Nicola Forcina, Giulia Norcia, Luigia Passamano, Marianna Scutifero, Alice Gardani, Antonella Pini, Giulia Monaco, Maria Grazia D'Angelo, Michele Giovanni D'Angelo Bozzi, Daniela Leone, Riccardo Zanin, Renata Zanin, Gian Luca Vita, Chiara Panicucci, Claudio Bruno, Tiziana Mongini, Federica Ricci, Angela Berardinelli, Roberta Battini, Riccardo Masson, Giovanni Baranello, Claudia Dosi, Enrico Bertini, Enrico Silvio Bertini, Vincenzo Nigro, Luisa Politano, Eugenio Maria Mercuri

Research output: Contribution to journalArticle

Abstract

Duchenne muscular dystrophy (DMD) is a neuromuscular condition characterized by muscle weakness. The Performance of upper limb (PUL) test is designed to evaluate upper limb function in DMD patients across three domains. The aim of this study is to identify frequently lost or gained PUL 2.0 abilities at distinct functional stages in DMD patients. This retrospective study analyzed prospectively collected data on 24-month PUL 2.0 changes related to ambulatory function. Ambulant patients were categorized based on initial 6MWT distance, non-ambulant patients by time since ambulation loss. Each PUL 2.0 item was classified as shift up, no change, or shift down. The study's cohort incuded 274 patients, with 626 paired evaluations at the 24-month mark. Among these, 55.1 % had activity loss, while 29.1 % had gains. Ambulant patients showed the lowest loss rates, mainly in the shoulder domain. The highest loss rate was in the shoulder domain in the transitioning subgroup and in elbow and distal domains in the non-ambulant patients. Younger ambulant patients demonstrated multiple gains, whereas in the other functional subgroups there were fewer gains, mostly tied to singular activities. Our findings highlight divergent upper limb domain progression, partly linked to functional status and baseline function.
Original languageEnglish
Pages (from-to)75-82
Number of pages8
JournalNeuromuscular Disorders
Volume34
DOIs
Publication statusPublished - 2024

Keywords

  • Duchenne muscular dystrophy
  • Performance of upper limb
  • Shift analysis
  • Upper limb disease progression
  • Upper limb function

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