Long-term Effects of Androgen Deprivation in a Patient with Spinal and Bulbar Muscular Atrophy - A Case Report with 14 Years of Follow-up
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- Hijikata Yasuhiro
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Hashizume Atsushi
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Yamada Shinichiro
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Ito Daisuke
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Banno Haruhiko
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Suzuki Keisuke
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan Innovation Center for Clinical Research, National Center for Geriatrics and Gerontology, Japan
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- Sobue Gen
- Research Division of Dementia and Neurodegenerative Disease, Nagoya University Graduate School of Medicine, Japan
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- Katsuno Masahisa
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Abstract
<p>Spinal and bulbar muscular atrophy (SBMA) is a progressive hereditary neuromuscular disease caused by the testosterone-dependent accumulation of pathogenic polyglutamine-expanded androgen receptor protein. A 41-year-old man with SBMA received the androgen deprivation agent leuprorelin acetate for 7 years in clinical trials and underwent castration following the trial. Suppression of testosterone levels for 14 years resulted in a slower disease progression, as measured prospectively with quantitative measurements, than the historical control data reported in previous studies. This suggests that long-term androgen deprivation delays disease progression in SBMA. </p>
Journal
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- Internal Medicine
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Internal Medicine 58 (15), 2231-2234, 2019-08-01
The Japanese Society of Internal Medicine