TY - JOUR
T1 - North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy
AU - Mazzone, Elena
AU - Martinelli, Diego
AU - Berardinelli, Angela
AU - Messina, Sonia
AU - D'Amico, Adele
AU - Vasco, Gessica
AU - Main, Marion
AU - Doglio, Luca
AU - Politano, Luisa
AU - Cavallaro, Filippo
AU - Frosini, Silvia
AU - Bello, Luca
AU - Carlesi, Adelina
AU - Bonetti, Anna Maria
AU - Zucchini, Elisabetta
AU - Sanctis, Roberto De
AU - Scutifero, Marianna
AU - Bianco, Flaviana
AU - Rossi, Francesca
AU - Motta, Maria Chiara
AU - Sacco, Annalisa
AU - Donati, Maria Alice
AU - Mongini, Tiziana
AU - Pini, Antonella
AU - Battini, Roberta
AU - Pegoraro, Elena
AU - Pane, Marika
AU - Pasquini, Elisabetta
AU - Bruno, Claudio
AU - Vita, Giuseppe
AU - Waure, Chiara de
AU - Bertini, Enrico
AU - Mercuri, Eugenio
PY - 2010/11
Y1 - 2010/11
N2 - The North Star Ambulatory Assessment is a functional scale specifically designed for ambulant boys affected by Duchenne muscular dystrophy (DMD). Recently the 6-minute walk test has also been used as an outcome measure in trials in DMD.The aim of our study was to assess a large cohort of ambulant boys affected by DMD using both North Star Assessment and 6-minute walk test. More specifically, we wished to establish the spectrum of findings for each measure and their correlation.This is a prospective multicentric study involving 10 centers. The cohort included 112 ambulant DMD boys of age ranging between 4.10 and 17. years (mean 8.18 ± 2.3 DS). Ninety-one of the 112 were on steroids: 37/91 on intermittent and 54/91 on daily regimen.The scores on the North Star assessment ranged from 6/34 to 34/34. The distance on the 6-minute walk test ranged from 127 to 560.6. m. The time to walk 10. m was between 3 and 15. s. The time to rise from the floor ranged from 1 to 27.5. s. Some patients were unable to rise from the floor.As expected the results changed with age and were overall better in children treated with daily steroids.The North Star assessment had a moderate to good correlation with 6-minute walk test and with timed rising from floor but less with 10. m timed walk/run test. The 6-minute walk test in contrast had better correlation with 10. m timed walk/run test than with timed rising from floor. These findings suggest that a combination of these outcome measures can be effectively used in ambulant DMD boys and will provide information on different aspects of motor function, that may not be captured using a single measure.
AB - The North Star Ambulatory Assessment is a functional scale specifically designed for ambulant boys affected by Duchenne muscular dystrophy (DMD). Recently the 6-minute walk test has also been used as an outcome measure in trials in DMD.The aim of our study was to assess a large cohort of ambulant boys affected by DMD using both North Star Assessment and 6-minute walk test. More specifically, we wished to establish the spectrum of findings for each measure and their correlation.This is a prospective multicentric study involving 10 centers. The cohort included 112 ambulant DMD boys of age ranging between 4.10 and 17. years (mean 8.18 ± 2.3 DS). Ninety-one of the 112 were on steroids: 37/91 on intermittent and 54/91 on daily regimen.The scores on the North Star assessment ranged from 6/34 to 34/34. The distance on the 6-minute walk test ranged from 127 to 560.6. m. The time to walk 10. m was between 3 and 15. s. The time to rise from the floor ranged from 1 to 27.5. s. Some patients were unable to rise from the floor.As expected the results changed with age and were overall better in children treated with daily steroids.The North Star assessment had a moderate to good correlation with 6-minute walk test and with timed rising from floor but less with 10. m timed walk/run test. The 6-minute walk test in contrast had better correlation with 10. m timed walk/run test than with timed rising from floor. These findings suggest that a combination of these outcome measures can be effectively used in ambulant DMD boys and will provide information on different aspects of motor function, that may not be captured using a single measure.
KW - 6-Minute walk test
KW - Duchenne muscular dystrophy
KW - North star ambulatory assessment
UR - http://www.scopus.com/inward/record.url?scp=77957748915&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77957748915&partnerID=8YFLogxK
U2 - 10.1016/j.nmd.2010.06.014
DO - 10.1016/j.nmd.2010.06.014
M3 - Article
C2 - 20634072
AN - SCOPUS:77957748915
VL - 20
SP - 712
EP - 716
JO - Neuromuscular Disorders
JF - Neuromuscular Disorders
SN - 0960-8966
IS - 11
ER -