title: The 6 minute walk test and performance of upper limb in ambulant duchenne muscular dystrophy boys.
abstract: The Performance of Upper Limb (PUL) test was specifically developed for the assessment of upper limbs in Duchenne muscular dystrophy (DMD). The first published data have shown that early signs of involvement can also be found in ambulant DMD boys. The aim of this longitudinal Italian multicentric study was to evaluate the correlation between the 6 Minute Walk Test (6MWT) and the PUL in ambulant DMD boys. Both 6MWT and PUL were administered to 164 ambulant DMD boys of age between 5.0 and 16.17 years (mean 8.82). The 6 minute walk distance (6MWD) ranged between 118 and 557 (mean: 376.38, SD: 90.59). The PUL total scores ranged between 52 and 74 (mean: 70.74, SD: 4.66). The correlation between the two measures was 0.499. The scores on the PUL largely reflect the overall impairment observed on the 6MWT but the correlation was not linear. The use of the PUL appeared to be less relevant in the very strong patients with 6MWD above 400 meters, who, with few exceptions had near full scores. In patients with lower 6MWD the severity of upper limb involvement was more variable and could not always be predicted by the 6MWD value or by the use of steroids. Our results confirm that upper limb involvement can already be found in DMD boys even in the ambulant phase.
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Marika
authors: M
authors: Pane
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Elena Stacy
authors: ES
authors: Mazzone
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Serena
authors: S
authors: Sivo
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Lavinia
authors: L
authors: Fanelli
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Roberto
authors: R
authors: De Sanctis
authors: Department of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, Rome, Italy.
authors: Adele
authors: A
authors: D'Amico
authors: Department of Neurosciences, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
authors: Sonia
authors: S
authors: Messina
authors: Department of Developmental Neuroscience, Stella Maris Institute, Pisa, Italy.
authors: Roberta
authors: R
authors: Battini
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Flaviana
authors: F
authors: Bianco
authors: Department of Experimental Medicine, Cardiomyology and Medical Genetics, Second University, Napoli, Italy.
authors: Marianna
authors: M
authors: Scutifero
authors: Department of Experimental Medicine, Cardiomyology and Medical Genetics, Second University, Napoli, Italy.
authors: Roberta
authors: R
authors: Petillo
authors: Department of Developmental Neuroscience, Stella Maris Institute, Pisa, Italy.
authors: Silvia
authors: S
authors: Frosini
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Roberta
authors: R
authors: Scalise
authors: Department of Neurosciences, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
authors: Gian Luca
authors: GL
authors: Vita
authors: Neuromuscular Disease Unit, Giannina Gaslini Institute, Genoa, Italy.
authors: Claudio
authors: C
authors: Bruno
authors: Neuromuscular Disease Unit, Giannina Gaslini Institute, Genoa, Italy.
authors: Marina
authors: M
authors: Pedemonte
authors: Neuromuscular Center, San Giovanni Battista Hospital, University of Turin, Turin, Italy.
authors: Tiziana
authors: T
authors: Mongini
authors: Department of Neurosciences, University of Padua, Padua, Italy.
authors: Elena
authors: E
authors: Pegoraro
authors: Child Neurology and Psychiatry Unit, "Casimiro Mondino" Foundation, Pavia, Italy.
authors: Francesca
authors: F
authors: Brustia
authors: Child Neurology and Psychiatry Unit, "Casimiro Mondino" Foundation, Pavia, Italy.
authors: Alice
authors: A
authors: Gardani
authors: Child Neurology and Psychiatry Unit, "Casimiro Mondino" Foundation, Pavia, Italy.
authors: Angela
authors: A
authors: Berardinelli
authors: Neuromuscular Center, San Giovanni Battista Hospital, University of Turin, Turin, Italy.
authors: Valentina
authors: V
authors: Lanzillotta
authors: Department of Experimental Medicine, Cardiomyology and Medical Genetics, Second University, Napoli, Italy.
authors: Emanuela
authors: E
authors: Viggiano
authors: Department of Neurosciences, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
authors: Filippo
authors: F
authors: Cavallaro
authors: Department of Neurosciences, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
authors: Maria
authors: M
authors: Sframeli
authors: Department of Neurosciences, University of Padua, Padua, Italy.
authors: Luca
authors: L
authors: Bello
authors: Department of Neurosciences, University of Padua, Padua, Italy.
authors: Andrea
authors: A
authors: Barp
authors: Department of Neurosciences, University of Padua, Padua, Italy.
authors: Fabio
authors: F
authors: Busato
authors: Child Neurology and Psychiatry Unit, Maggiore Hospital, Bologna, Italy.
authors: Serena
authors: S
authors: Bonfiglio
authors: Centro Clinico Nemo, Milan, Italy.
authors: Enrica
authors: E
authors: Rolle
authors: Department of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, Rome, Italy.
authors: Giulia
authors: G
authors: Colia
authors: Department of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, Rome, Italy.
authors: Annamaria
authors: A
authors: Bonetti
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Concetta
authors: C
authors: Palermo
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Alessandra
authors: A
authors: Graziano
authors: IRCCS Eugenio Medea, Bosisio Parini, Italy.
authors: Grazia
authors: G
authors: D'Angelo
authors: Child Neurology and Psychiatry Unit, Maggiore Hospital, Bologna, Italy.
authors: Antonella
authors: A
authors: Pini
authors: Developmental Neurology Unit, Neurological InstituteCarlo Besta, Milan, Italy.
authors: Alice
authors: A
authors: Corlatti
authors: Centro Clinico Nemo, Milan, Italy.
authors: Ksenija
authors: K
authors: Gorni
authors: Developmental Neurology Unit, Neurological InstituteCarlo Besta, Milan, Italy.
authors: Giovanni
authors: G
authors: Baranello
authors: Pediatric Neurology and Psychiatry Unit, Sapienza University, Rome, Italy.
authors: Laura
authors: L
authors: Antonaci
authors: Department of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, Rome, Italy.
authors: Enrico
authors: E
authors: Bertini
authors: Department of Experimental Medicine, Cardiomyology and Medical Genetics, Second University, Napoli, Italy.
authors: Luisa
authors: L
authors: Politano
authors: Department of Paediatric Neurology, Catholic University, Rome, Italy.
authors: Eugenio
authors: E
authors: Mercuri
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