Long-term Effects of Androgen Deprivation in a Patient with Spinal and Bulbar Muscular Atrophy - A Case Report with 14 Years of Follow-up

  • Hijikata Yasuhiro
    Department of Neurology, Nagoya University Graduate School of Medicine, Japan
  • Hashizume Atsushi
    Department of Neurology, Nagoya University Graduate School of Medicine, Japan
  • Yamada Shinichiro
    Department of Neurology, Nagoya University Graduate School of Medicine, Japan
  • Ito Daisuke
    Department of Neurology, Nagoya University Graduate School of Medicine, Japan
  • Banno Haruhiko
    Department of Neurology, Nagoya University Graduate School of Medicine, Japan
  • Suzuki Keisuke
    Department of Neurology, Nagoya University Graduate School of Medicine, Japan Innovation Center for Clinical Research, National Center for Geriatrics and Gerontology, Japan
  • Sobue Gen
    Research Division of Dementia and Neurodegenerative Disease, Nagoya University Graduate School of Medicine, Japan
  • 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

  • Internal Medicine

    Internal Medicine 58 (15), 2231-2234, 2019-08-01

    The Japanese Society of Internal Medicine

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