{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T09:23:53Z","timestamp":1789982633843,"version":"4.0.1"},"reference-count":31,"publisher":"BMJ","issue":"12","funder":[{"name":"grants from Japan Agency for Medical Research and Development"},{"name":"Grant-in-Aids (KAKENHI) from Ministry of Education, Culature, Sports, Science, and Technology of Japan"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1136\/jnnp-2017-316015","type":"journal-article","created":{"date-parts":[[2017,8,6]],"date-time":"2017-08-06T00:30:12Z","timestamp":1501979412000},"page":"1026-1032","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":49,"title":["Long-term treatment with leuprorelin for spinal and bulbar muscular atrophy: natural history-controlled study"],"prefix":"10.1136","volume":"88","author":[{"given":"Atsushi","family":"Hashizume","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahisa","family":"Katsuno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisuke","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiro","family":"Hirakawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiro","family":"Hijikata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichiro","family":"Yamada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonori","family":"Inagaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruhiko","family":"Banno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gen","family":"Sobue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2017,8,5]]},"reference":[{"key":"key-10.1136\/jnnp-2017-316015-1","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1212\/WNL.18.7.671","article-title":"Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait","volume":"18","author":"Kennedy","year":"1968","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2017-316015-2","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1093\/brain\/112.1.209","article-title":"X-linked recessive bulbospinal neuronopathy. A clinicopathological study","volume":"112","author":"Sobue","year":"1989","journal-title":"Brain"},{"key":"key-10.1136\/jnnp-2017-316015-3","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1001\/archneur.59.12.1921","article-title":"X-linked bulbospinal neuronopathy: kennedy disease","volume":"59","author":"Sperfeld","year":"2002","journal-title":"Arch Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-4","doi-asserted-by":"crossref","first-page":"1446","DOI":"10.1093\/brain\/awl096","article-title":"Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 japanese patients","volume":"129","author":"Atsuta","year":"2006","journal-title":"Brain"},{"key":"key-10.1136\/jnnp-2017-316015-5","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/j.pneurobio.2012.05.007","article-title":"Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA)","volume":"99","author":"Katsuno","year":"2012","journal-title":"Prog Neurobiol"},{"key":"key-10.1136\/jnnp-2017-316015-6","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1111\/odi.12121","article-title":"Spinal and bulbar muscular atrophy: pathogenesis and clinical management","volume":"20","author":"Grunseich","year":"2014","journal-title":"Oral Dis"},{"key":"key-10.1136\/jnnp-2017-316015-7","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/352077a0","article-title":"Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy","volume":"352","author":"La Spada","year":"1991","journal-title":"Nature"},{"key":"key-10.1136\/jnnp-2017-316015-8","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1016\/S0896-6273(02)00834-6","article-title":"Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"35","author":"Katsuno","year":"2002","journal-title":"Neuron"},{"key":"key-10.1136\/jnnp-2017-316015-9","doi-asserted-by":"crossref","first-page":"2663","DOI":"10.1172\/JCI28773.","article-title":"Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model","volume":"116","author":"Yu","year":"2006","journal-title":"J Clin Invest"},{"key":"key-10.1136\/jnnp-2017-316015-10","doi-asserted-by":"crossref","first-page":"4778","DOI":"10.1523\/JNEUROSCI.0808-04.2004","article-title":"Castration restores function and neurofilament alterations of aged symptomatic males in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"24","author":"Chevalier-Larsen","year":"2004","journal-title":"J Neurosci"},{"key":"key-10.1136\/jnnp-2017-316015-11","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1038\/nm878","article-title":"Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy","volume":"9","author":"Katsuno","year":"2003","journal-title":"Nat Med"},{"key":"key-10.1136\/jnnp-2017-316015-12","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1002\/ana.21540","article-title":"Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy","volume":"65","author":"Banno","year":"2009","journal-title":"Ann Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-13","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1016\/S1474-4422(10)70182-4","article-title":"Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial","volume":"9","author":"Katsuno","year":"2010","journal-title":"Lancet Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-14","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/S1474-4422(10)70321-5","article-title":"Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: a randomised placebo-controlled trial","volume":"10","author":"Fern\u00e1ndez-Rhodes","year":"2011","journal-title":"Lancet Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-15","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1136\/jnnp-2011-301307","article-title":"Perspectives on molecular targeted therapies and clinical trials for neurodegenerative diseases","volume":"83","author":"Katsuno","year":"2012","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2017-316015-16","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1002\/ana.20735","article-title":"Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker","volume":"59","author":"Banno","year":"2006","journal-title":"Ann Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-17","doi-asserted-by":"crossref","first-page":"2838","DOI":"10.1093\/brain\/aws170","article-title":"Longitudinal changes of outcome measures in spinal and bulbar muscular atrophy","volume":"135","author":"Hashizume","year":"2012","journal-title":"Brain"},{"key":"key-10.1136\/jnnp-2017-316015-18","first-page":"1343","article-title":"[Clinical phase I and phase II study on a sustained release formulation of leuprorelin acetate (TAP-144-SR), an LH-RH agonist, in patients with prostatic carcinoma. collaborative++ Studies on Prostatic Carcinoma by the Study Group for TAP-144-SR]","volume":"36","author":"Niijima","year":"1990","journal-title":"Hinyokika Kiyo"},{"key":"key-10.1136\/jnnp-2017-316015-19","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1159\/000474238","article-title":"Randomized open labelled comparative study of the efficacy, safety and tolerability of leuprorelin acetate 1M and 3M depot in patients with advanced prostatic cancer","volume":"30 Suppl 1","author":"Wechsel","year":"1996","journal-title":"Eur Urol"},{"key":"key-10.1136\/jnnp-2017-316015-20","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1001\/archneur.1996.00550020045014","article-title":"The ALS CNTF treatment study (ACTS) phase \u2160-\u2161 Study Group. The Amyotrophic Lateral Sclerosis Functional Rating Scale. Assessment of activities of daily living in patients with amyotrophic lateral sclerosis","volume":"53","year":"1996","journal-title":"Arch Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-21","first-page":"346","article-title":"[Study of functional rating scale for amyotrophic lateral sclerosis: revised ALSFRS (ALSFRS-R) Japanese version]","volume":"53","author":"Ohashi","year":"2001","journal-title":"No To Shinkei"},{"key":"key-10.1136\/jnnp-2017-316015-22","first-page":"999","article-title":"[Reliability and factorial structure of a rating scale for amyotrophic lateral sclerosis]","volume":"48","author":"Oda","year":"1996","journal-title":"No To Shinkei"},{"key":"key-10.1136\/jnnp-2017-316015-23","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1002\/ana.410320517","article-title":"Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene","volume":"32","author":"Doyu","year":"1992","journal-title":"Ann Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-24","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.nbd.2006.12.015","article-title":"Lost in translation: treatment trials in the SOD1 mouse and in human ALS","volume":"26","author":"Benatar","year":"2007","journal-title":"Neurobiol Dis"},{"key":"key-10.1136\/jnnp-2017-316015-25","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1016\/S1474-4422(14)70129-2","article-title":"Clinical trials in amyotrophic lateral sclerosis: why so many negative trials and how can trials be improved?","volume":"13","author":"Mitsumoto","year":"2014","journal-title":"Lancet Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-26","volume-title":"Rare diseases: common issues in Drug Development Guidance for Industry. Draft guidance","author":"U.S. Department of Health and Human Services Food and Drug Administration, Center for Drug evaluation and research (CDER), Center for Biologics evaluation and research (CBER)","year":"2015"},{"key":"key-10.1136\/jnnp-2017-316015-27","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1001\/jamaneurol.2015.4885","article-title":"Clinical Trial Simulations Based on Genetic Stratification and the Natural History of a Functional Outcome Measure in Creutzfeldt-Jakob Disease","volume":"73","author":"Mead","year":"2016","journal-title":"JAMA Neurol"},{"key":"key-10.1136\/jnnp-2017-316015-28","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1111\/1754-9485.12124","article-title":"Effect of androgen deprivation therapy on muscle attenuation in men with prostate Cancer","volume":"58","author":"Chang","year":"2014","journal-title":"J Med Imaging Radiat Oncol"},{"key":"key-10.1136\/jnnp-2017-316015-29","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1212\/01.wnl.0000324635.49971.3d","article-title":"The placebo treatments in neurosciences: new insights from clinical and neuroimaging studies","volume":"71","author":"Diederich","year":"2008","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2017-316015-30","doi-asserted-by":"crossref","first-page":"S51","DOI":"10.1212\/WNL.0b013e318199049e","article-title":"\u201cDisease-modification\u201d\u00a0trials in Parkinson disease: target populations, endpoints and study design","volume":"72","author":"Rascol","year":"2009","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2017-316015-31","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1007\/s00415-011-6251-2","article-title":"Difference in chronological changes of outcome measures between untreated and placebo-treated patients of spinal and bulbar muscular atrophy","volume":"259","author":"Hashizume","year":"2012","journal-title":"J Neurol"}],"container-title":["Journal of Neurology, Neurosurgery &amp; Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jnnp-2017-316015","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,25]],"date-time":"2020-09-25T18:07:59Z","timestamp":1601057279000},"score":1,"resource":{"primary":{"URL":"https:\/\/jnnp.bmj.com\/lookup\/doi\/10.1136\/jnnp-2017-316015"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,8,5]]},"references-count":31,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2017,11,28]]},"published-print":{"date-parts":[[2017,12]]}},"alternative-id":["10.1136\/jnnp-2017-316015"],"URL":"https:\/\/doi.org\/10.1136\/jnnp-2017-316015","relation":{},"ISSN":["0022-3050","1468-330X"],"issn-type":[{"value":"0022-3050","type":"print"},{"value":"1468-330X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,8,5]]}}}