INTRODUCTION: We investigated the long-term effects of leuprorelin on leg-muscle strength in spinal and bulbar muscular atrophy (SBMA). We hypothesized that testosterone suppression by leuprorelin would prevent the progression of muscle weakness. METHODS: In a prospective, long duration, open trial, 16 SBMA patients underwent medical castration with leuprorelin for 3.5 years. Chlormadinone was coadministered initially to prevent a testosterone surge. The strength of knee extension and flexion were quantitated using a torque machine. RESULTS: Our hypothesis was rejected. The leg strength measures decreased significantly with the mean reduction of 22.3-27.8%. In a post hoc analysis, the leg strength of 4 patients with higher pretreatment baseline total testosterone levels and short disease duration of 1-6 years were stronger at baseline and decreased by only 12.3-15.7% after treatment. CONCLUSIONS: Leuprorelin was not effective in this small long-term treatment trial in SBMA. The possibility that earlier treatment might be beneficial may deserve further study.
INTRODUCTION: We investigated the long-term effects of leuprorelin on leg-muscle strength in spinal and bulbar muscular atrophy (SBMA). We hypothesized that testosterone suppression by leuprorelin would prevent the progression of muscle weakness. METHODS: In a prospective, long duration, open trial, 16 SBMApatients underwent medical castration with leuprorelin for 3.5 years. Chlormadinone was coadministered initially to prevent a testosterone surge. The strength of knee extension and flexion were quantitated using a torque machine. RESULTS: Our hypothesis was rejected. The leg strength measures decreased significantly with the mean reduction of 22.3-27.8%. In a post hoc analysis, the leg strength of 4 patients with higher pretreatment baseline total testosterone levels and short disease duration of 1-6 years were stronger at baseline and decreased by only 12.3-15.7% after treatment. CONCLUSIONS: Leuprorelin was not effective in this small long-term treatment trial in SBMA. The possibility that earlier treatment might be beneficial may deserve further study.
Authors: Lori Zboray; Anna Pluciennik; Dana Curtis; Yuhong Liu; Lisa D Berman-Booty; Christopher Orr; Cristina T Kesler; Tamar Berger; Daniel Gioeli; Bryce M Paschal; Diane E Merry Journal: Cell Rep Date: 2015-12-07 Impact factor: 9.423
Authors: Tiffany W Todd; Hiroshi Kokubu; Helen C Miranda; Constanza J Cortes; Albert R La Spada; Janghoo Lim Journal: Elife Date: 2015-08-26 Impact factor: 8.140