题名

甘迺迪氏症與性功能：最新系統性文獻回顧

并列篇名

Kennedy Disease and Sexual Function: An Updated Systematic Review of Literature

DOI

10.6206/SIS.202007_11(1).0003

作者

高宜君(I-Chun Kao)；林宛瑾(Wan-Chin Lin)；陳偉熹(Wei-Hsi Chen)

关键词

甘迺迪氏症 ； 性欲 ； 勃起功能 ； 雄激素 ； 雄激素接受器 ； Kennedy Disease ； Sexual Desire ； Erectile Function ； Androgen ； Androgen Receptor

期刊名称

性學研究

卷期/出版年月

11卷1期（2020 / 07 / 01）

页次

45 - 62

内容语文

繁體中文
中文摘要

甘迺迪氏症是X染色體上雄激素接受器基因表現異常，導致雄激素接受器不敏感，而產生神經系統和其他系統功能障礙之遺傳性疾病。直至目前，並沒有正式的系統性文獻回顧去瞭解甘迺迪氏症的性功能變化，故無法提供足夠的科學證據完成性功能諮商。本文之目的，是透過國際醫學資料庫搜尋相關文獻並整理結果，瞭解甘迺迪氏症的性功能障礙比例和類型。結果共有16篇相關研究合計374位男性，雖然文獻水平幾乎為第三或四級，仍然提供寶貴的訊息。分析顯示性功能障礙隨著疾病病程發展漸漸出現，在身體肌肉無力症狀出現後平均約七年內發生。性交滿意度、整體滿意度、高潮功能、勃起功能、性欲功能較同年齡層男性皆有不同程度之下降，尤其是勃起功能和性欲的影響更為常見。因此，性功能障礙在甘迺迪氏症中並非少見，建議在帶原者、無症狀者和早期症狀者即予以完整的性功能諮商。
英文摘要

Kennedy disease is a X-linked hereditary disease of the androgen receptor in that insensitivity of androgen generates a variety of neurological and systemic disorders. Till now, there is no formal systematic review concerning the sexual function of Kennedy disease to provide sufficient scientific information for sexual function counseling. The aim of this study was to search the relationship between Kennedy disease and sexual function from medical database to understand the frequency and types of sexual dysfunction in persons with Kennedy disease. Finally, there were a total of 16 publications, including 374 men, enrolled in this study. Although the evidences were almost the III or IV level, these studies still provided noteworthy information. The results showed that sexual dysfunction progressively appeared during the course of disease and usually within 7 years after weakness occurred. The intercourse satisfaction, overall satisfaction, orgasmic function, erectile function, and sexual desire variably decreased. The erectile function and sexual desire were especially more commonly involved. Therefore, sexual dysfunction is not uncommon in persons with Kennedy disease. Complete sexual function counseling is suggested for carrier, asymptomatic persons, and persons with early stage of manifestations.
主题分类 社會科學 > 社會學
参考文献
  1. Alves, C. N.,Braga, T. K. K.,Somensi, D. N.,Nascimento, B. S.,de Lima, J. A. S.,Fujihara, S.(2018).X-linked spinal and bulbar muscular atrophy (Kennedy’s disease): The first case described in the Brazilian Amazon.Einstein (São Paulo),16(2),eRC4011.
  2. American Psychiatric Association,徐翊健（譯）,高廉程（譯）,張杰（譯）,葉大全（譯）,黃郁潔（譯）,黃鈺蘋（譯）,劉佑閔（譯）(2018).DSM-5精神疾病診斷與統計.台北市:合記.
  3. Appel, J. M.(2010).Sex rights for the disabled?.Journal of Medical Ethics,36,152-154.
  4. Arbizu, T.,Santamaría, J.,Gomez, J. M.,Quílez, A.,Serra, J. P.(1983).A family with adult spinal and bulbar muscular atrophy, X-linked inheritance and associated testicular failure.Journal of the Neurological Sciences,59,371-382.
  5. Bahner, J.(2020).Sexual citizenship and disability: Understanding sexual support in policy, practice and theory.New York:Routledge.
  6. Baniahmad, A.(2015).Inhibition of the androgen receptor by antiandrogens in spinobulbar muscle atrophy.Journal of Molecular Neuroscience,58,343-347.
  7. Banno, H.,Katsuno, M.,Suzuki, K.,Tanaka, F.,Sobue, G.(2012).Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy (SBMA).Cell and Tissue Research,349,313-320.
  8. Breza, M.,Koutsis, G.(2018).Kennedy’s disease (spinal and bulbar muscular atrophy): A clinically oriented review of a rare disease.Journal of Neurology,266,565-573.
  9. Burnett, A. L.,Nehra, A.,Breau, R. H.,Culkin, D. J.,Faraday, M. M.,Hakim, L. S.,Shindel, A. W.(2018).Erectile dysfunction: AUA guideline.The Journal of Urology,200,633-641.
  10. Care Quality Commission. (2019). Relationships and sexuality in adult social care services: Guidance for CQC inspection staff and registered adult social care providers. Retrieved April 7, 2017, from https://www.cqc.org.uk/sites/default/files/20190221-Relationships-and-sexuality-in-social-care-PUBLICATION.pdf
  11. Chen, L. W.-H.,Chen, M. Y.-S.,Chen, K.-Y.,Lin, H.-S.,Chien, C.-C.,Yin, H. -L.(2017).Topiramate-associated sexual dysfunction: A systematic review.Epilepsy & Behavior,73,10-17.
  12. Chen, L. W.-H.,Yin, H.-L.(2017).A literature review of antithrombotic and anticoagulating agents on sexual function.Andrologia,49,e12784.
  13. Chen, Y.,Luo, P.,Li, Z.,Hu, H.,Wu, D.,Xu, T.,Xie, H.(2017).Kennedy disease with difficulty in differential diagnosis: A case report.Medicine,96,e6792.
  14. Crewe, N. M.,Krause, J. S.(1988).Marital relationships and spinal cord injury.Archives of Physical Medicine & Rehabilitation,69,435-438.
  15. DeLoach, C. P.(1994).Attitudes toward disability: Impact on sexual development and forging of intimate relationships.Journal of Applied Rehabilitation Counselling,25(1),18-25.
  16. Di Giulio, G.(2003).Sexuality and people living with physical or developmental disabilities: A review of key issues.The Canadian Journal of Human Sexuality,12,53-68.
  17. Di Nucci, E.(2011).Sexual rights and disability.Journal of Medical Ethics,37,158-161.
  18. Dinsmore, W. W.,Hodges, M.,Hargreaves, C.,Osterloh, I. H.,Smith, M. D.,Rosen, R. C.(1999).Sildenafil citrate (Viagra) in erectile dysfunction: Near normalization in men with broad-spectrum erectile dysfunction compared with age-matched healthy control subjects.Urology,53,800-805.
  19. Dubey, A.,Jain, R.,Sodani, A. K.,Chouksey, D.(2017).A case of Kennedy’s disease from India.Annals of Indian Academy of Neurology,20,163-164.
  20. Fernández-Rhodes, L. E.,Kokkinis, A. D.,White, M. J.,Watts, C. A.,Auh, S.,Jeffries, N. O.,Fischbeck, K. H.(2011).Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: A randomised placebo-controlled trial.The Lancet Neurology,10(10),140-147.
  21. Finsterer, J.,Scorza, F. A.(2019).Central nervous system abnormalities in spinal and bulbar muscular atrophy (Kennedy’s disease).Clinical Neurology and Neurosurgery,184,105426.
  22. Finsterer, J.,Soraru, G.(2015).Onset manifestations of spinal and bulbar muscular atrophy (Kennedy’s disease).Journal of Molecular Neuroscience,58,321-329.
  23. Foster, L. A.,Salajegheh, M. K.(2019).Motor neuron disease: Pathophysiology, diagnosis, and management.The American Journal of Medicine,132,32-37.
  24. Fratta, P.,Nirmalananthan, N.,Masset, L.,Skorupinska, I.,Collins, T.,Cortese, A.,Hanna, M. G.(2014).Correlation of clinical and molecular features in spinal bulbar muscular atrophy.Neurology,82,2077-2084.
  25. Fu, S.,Kuo, H.,Chu, C.,Wu, Y.,Ro, L.,Liu, C.,Huang, C.(2013).Long-term follow-up of spinal and bulbar muscular atrophy in Taiwan.Journal of the Formosan Medical Association,112,326-331.
  26. Grunseich, C.,Kats, I. R.,Bott, L. C.,Rinaldi, C.,Kokkinis, A.,Fox, D.,Fischbeck, K. H.(2014).Early onset and novel features in a spinal and bulbar muscular atrophy patient with a 68 CAG repeat.Neuromuscular Disorders,24,978-981.
  27. Guidetti, D.,Motti, L.,Marcello, N.,Vescovini, E.,Marbini, A.,Dotti, C.,Solimè, F.(1986).Kennedy disease in an Italian kindred.European Neurology,25,188-196.
  28. Gulía, C.,Baldassarra, S.,Zangari, A.,Briganti, V.,Gigli, S.,Gaffi, M.,Piergentili, R.(2018).Androgen insensitivity syndrome.European Review for Medical and Pharmacological Sciences,22,3873-3887.
  29. Harutunian, G. M.,Beydoun, S. R.,Rison, R. A.(2013).Kennedy disease misdiagnosed as polymyositis: A case report.BMC Research Notes,6,389.
  30. Hashizume, A.,Katsuno, M.,Suzuki, K.,Banno, H.,Takeuchi, Y.,Kawashima, M.,Sobue, G.(2019).Efficacy and safety of leuprorelin acetate for subjects with spinal and bulbar muscular atrophy: Pooled analyses of two randomizedcontrolled trials.Journal of Neurology,266,1211-1221.
  31. Hausmanowa-Petrusewicz, I.,Borkowska, J.,Janczewski, Z.(1983).X-linked adult form of spinal muscular atrophy.Journal of Neurology,229,175-188.
  32. Jokelainen, M.,Palo, J.(1976).Amyotrophic lateral sclerosis and autonomic nervous system.The Lancet,307,1246.
  33. Kassubek, J.,Juengling, F. D.,Sperfeld, A.-D.(2007).Widespread white matter changes in Kennedy disease: A voxel based morphometry study.Journal of Neurology, Neurosurgery & Psychiatry,78,1209-1212.
  34. Kennedy, W. R.,Alter, M.,Sung, J. H.(1968).Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.Neurology,18,671-680.
  35. Kessler, H.,Prudlo, J.,Kraft, S.,Supprian, T.(2005).Dementia of frontal lobe type in Kennedy’s disease.Amyotrophic Lateral Sclerosis,6,250-253.
  36. Kreuter, M.,Sullivan, M.,Siösteen, A.(1994).Sexual adjustment after spinal cord injury-comparison of partner experiences in pre- and postinjury relationships.Spinal Cord,32,759-770.
  37. Li, F.,Aji, G.,Leng, F.,Chen, J.-C.,Luo, Y.,Zhang, J.,Lu, Z.(2018).Endocrinal description of two Chinese Kennedy’s disease pedigrees.Journal of Clinical Neuroscience,47,245-248.
  38. Milligan, M. S.,Neufeldt, A. M.(2001).The myth of asexuality: A survey of social and empirical evidence.Sexuality and Disability,19,91-109.
  39. Motor Neurone Disease Association. (2010). Sex and relationships: For partners of people living with MND. Retrieved April 8, 2020, from https://alsworldwide.org/assets/misc/Information_sheet_20B_-_Sex_and_relationships_for_partners_of_people_living_with_MND.pdf
  40. Noggle, C. A.,Dean, R. S.,Horton, A. M., Jr.(2012).The encyclopedia of neuropsychological disorders.New York:Springer.
  41. Querin, G.,Sorarù, G.,Pradat, P.-F.(2017).Kennedy disease (X-linked recessive bulbospinal neuronopathy): A comprehensive review from pathophysiology to therapy.Revue Neurologique,173,326-337.
  42. Rhodes, L. E.,Freeman, B. K.,Auh, S.,Kokkinis, A. D.,La Pean, A.,Chen, C.,Fischbeck, K. H.(2009).Clinical features of spinal and bulbar muscular atrophy.Brain,132,3242-3251.
  43. Rinaldi, C.,Bott, L. C.,Fischbeck, K. H.(2014).Muscle matters in Kennedy’s disease.Neuron,82,251-253.
  44. Ringel, S. P.,Lava, N. S.,Treihaft, M. M.,Lubs, M. L.,Lubs, H. A.(1978).Late-onset X-linked recessive spinal and bulbar muscular atrophy.Muscle & Nerve,1,297-307.
  45. Shuttleworth, R. P.,Wedgwood, N.(2012).Disability, doctors and sexuality: Do healthcare providers influence the sexual wellbeing of people living with a neuromuscular disorder?.Sexuality and Disability,30,171-185.
  46. Sperfeld, A. D.,Karitzky, J.,Brummer, D.,Schreiber, H.,Häussler, J.,Ludolph, A. C.,Hanemann, C. O.(2002).X-linked bulbospinal neuronopathy: Kennedy disease.Archives of Neurology,59,1921-1926.
  47. Unrath, A.,Müller, H.-P.,Riecker, A.,Ludolph, A. C.,Sperfeld, A.-D.,Kassubek, J.(2010).Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging.Human Brain Mapping,31,1727-1740.
  48. Warrier, M. G.,Sadasivan, A.,Polavarapu, K.,Kumar, V. P.,Mahajan, N. P.,Reddy, C. P. C.,Thomas, P. T.(2020).Lived experience of spouses of persons with motor neuron disease: Preliminary findings through interpretative phenomenological analysis.Indian Journal of Palliative Care,26,60-65.
  49. 衛生福利部長期照顧司（2019）。公告修訂「長期照護醫師意見書」格式。上網日期：2020年4月8日，檢自https://dep.mohw.gov.tw/DOLTC/cp-4172-48916-123.html
print cancel