The aim of this study was to establish the possible effect of glucocorticoid treatment on upper limb function in a cohort of 91 non-ambulant DMD boys and adults of age between 11 and 26 years.All 91 were assessed using the Performance of Upper Limb test. Forty-eight were still on glucocorticoid after loss of ambulation, 25 stopped steroids at the time they lost ambulation and 18 were GC naïve or had steroids while ambulant for less than a year.At baseline the total scores ranged between 0 and 74 (mean 41.20). The mean total scores were 47.92 in the glucocorticoid group, 36 in those who stopped at loss of ambulation and 30.5 in the naïve group (p < 0.001).The 12-month changes ranged between -20 and 4 (mean -4.4). The mean changes were -3.79 in the glucocorticoid group, -5.52 in those who stopped at loss of ambulation and -4.44 in the naïve group. This was more obvious in the patients between 12 and 18 years and at shoulder and elbow levels.Our findings suggest that continuing glucocorticoids throughout teenage years and adulthood after loss of ambulation appears to have a beneficial effect on upper limb function.

Pane, M., Fanelli, L., Mazzone, E. S., Olivieri, G., D'Amico, A., Messina, S., Scutifero, M., Battini, R., Petillo, R., Frosini, S., Sivo, S., Vita, G. L., Bruno, C., Mongini, T., Pegoraro, E., De Sanctis, R., Gardani, A., Berardinelli, A., Lanzillotta, V., Carlesi, A., Viggiano, E., Cavallaro, F., Sframeli, M., Bello, L., Barp, A., Bianco, F., Bonfiglio, S., Rolle, E., Palermo, F. C., D'Angelo, G., Pini, A., Iotti, E., Gorni, K., Baranello, G., Bertini, E. S., Politano, L., Sormani, M. P., Mercuri, E. M., Benefits of glucocorticoids in non-ambulant boys/men with Duchenne muscular dystrophy: A multicentric longitudinal study using the Performance of Upper Limb test, <<NEUROMUSCULAR DISORDERS>>, 2015; 25 (10): 749-753. [doi:10.1016/j.nmd.2015.07.009] [https://hdl.handle.net/10807/260300]

Benefits of glucocorticoids in non-ambulant boys/men with Duchenne muscular dystrophy: A multicentric longitudinal study using the Performance of Upper Limb test

Pane, Marika;Mazzone, Elena Stacy;Olivieri, Giorgia;Battini, Roberta;Sivo, Serena;De Sanctis, Roberto;Cavallaro, Fabio;Bianco, Flaviana;Palermo, Francesco Cesare;Baranello, Giovanni;Bertini, Enrico Silvio;Mercuri, Eugenio Maria
2015

Abstract

The aim of this study was to establish the possible effect of glucocorticoid treatment on upper limb function in a cohort of 91 non-ambulant DMD boys and adults of age between 11 and 26 years.All 91 were assessed using the Performance of Upper Limb test. Forty-eight were still on glucocorticoid after loss of ambulation, 25 stopped steroids at the time they lost ambulation and 18 were GC naïve or had steroids while ambulant for less than a year.At baseline the total scores ranged between 0 and 74 (mean 41.20). The mean total scores were 47.92 in the glucocorticoid group, 36 in those who stopped at loss of ambulation and 30.5 in the naïve group (p < 0.001).The 12-month changes ranged between -20 and 4 (mean -4.4). The mean changes were -3.79 in the glucocorticoid group, -5.52 in those who stopped at loss of ambulation and -4.44 in the naïve group. This was more obvious in the patients between 12 and 18 years and at shoulder and elbow levels.Our findings suggest that continuing glucocorticoids throughout teenage years and adulthood after loss of ambulation appears to have a beneficial effect on upper limb function.
2015
Inglese
Pane, M., Fanelli, L., Mazzone, E. S., Olivieri, G., D'Amico, A., Messina, S., Scutifero, M., Battini, R., Petillo, R., Frosini, S., Sivo, S., Vita, G. L., Bruno, C., Mongini, T., Pegoraro, E., De Sanctis, R., Gardani, A., Berardinelli, A., Lanzillotta, V., Carlesi, A., Viggiano, E., Cavallaro, F., Sframeli, M., Bello, L., Barp, A., Bianco, F., Bonfiglio, S., Rolle, E., Palermo, F. C., D'Angelo, G., Pini, A., Iotti, E., Gorni, K., Baranello, G., Bertini, E. S., Politano, L., Sormani, M. P., Mercuri, E. M., Benefits of glucocorticoids in non-ambulant boys/men with Duchenne muscular dystrophy: A multicentric longitudinal study using the Performance of Upper Limb test, <<NEUROMUSCULAR DISORDERS>>, 2015; 25 (10): 749-753. [doi:10.1016/j.nmd.2015.07.009] [https://hdl.handle.net/10807/260300]
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