{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T14:51:35Z","timestamp":1790175095725,"version":"4.1.0"},"reference-count":27,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2018,10,10]],"date-time":"2018-10-10T00:00:00Z","timestamp":1539129600000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001807","name":"S\u00e3o Paulo Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001807","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001807","name":"FAPESP","doi-asserted-by":"publisher","award":["2013\/01766\u20107"],"award-info":[{"award-number":["2013\/01766\u20107"]}],"id":[{"id":"10.13039\/501100001807","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003593","name":"National Council for Scientific and Technological Development","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003593","name":"CNPq","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background<\/jats:bold>: Molecular imaging has proven to be a powerful tool to elucidate degenerated paths in a wide variety of neurological diseases and has not been systematically studied in hereditary spastic paraplegias.<\/jats:p><jats:p><jats:bold>Objectives<\/jats:bold>: To investigate dopaminergic degeneration in a cohort of 22 patients with hereditary spastic paraplegia attributed to <jats:italic>SPG11<\/jats:italic> mutations and evaluate treatment response to <jats:sc>l\u2010<\/jats:sc>dopa.<\/jats:p><jats:p><jats:bold>Methods<\/jats:bold>: Patients and controls underwent single\u2010photon emission computed tomography imaging utilizing <jats:sup>99m<\/jats:sup>Tc\u2010TRODAT\u20101 tracer. A single\u2010blind trial with 600\u2009mg of <jats:sc>l\u2010<\/jats:sc>dopa was performed comparing UPDRS scores.<\/jats:p><jats:p><jats:bold>Results<\/jats:bold>: Reduced dopamine transporter density was universal among patients. Nigral degeneration was symmetrical and correlated with disease duration and motor and cognitive handicap. No statistically significant benefit could be demonstrated with <jats:sc>l\u2010<\/jats:sc>dopa intake during the trial.<\/jats:p><jats:p><jats:bold>Conclusion<\/jats:bold>: Disruption of presynaptic dopaminergic pathways is a widespread phenomenon in patients with <jats:italic>SPG11<\/jats:italic> mutations, even in the absence of parkinsonism. Unresponsiveness to treatment could be related to postsynaptic damage that needs to be further investigated.<\/jats:p>","DOI":"10.1002\/mds.27491","type":"journal-article","created":{"date-parts":[[2018,10,11]],"date-time":"2018-10-11T00:17:49Z","timestamp":1539217069000},"page":"1650-1656","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["SPG11\u2010related parkinsonism: Clinical profile, molecular imaging and <scp>l<\/scp>\u2010dopa response"],"prefix":"10.1002","volume":"33","author":[{"given":"Ingrid","family":"Faber","sequence":"first","affiliation":[{"name":"Department of Neurology University of Campinas (UNICAMP) Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto Rolim Muro","family":"Martinez","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Campinas (UNICAMP) Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Carlos Roberto","family":"Martins","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Campinas (UNICAMP) Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maidane Luise","family":"Maia","sequence":"additional","affiliation":[{"name":"Division of Nuclear Medicine, Department of Radiology University of Campinas (UNICAMP)  Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliana Pasquotto","family":"Souza","sequence":"additional","affiliation":[{"name":"Division of Nuclear Medicine, Department of Radiology University of Campinas (UNICAMP)  Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles Marques","family":"Louren\u00e7o","sequence":"additional","affiliation":[{"name":"Department of Neurology University of S\u00e3o Paulo (USP\u2010RP)  Ribeir\u00e3o Preto Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Wilson","family":"Marques","sequence":"additional","affiliation":[{"name":"Department of Neurology University of S\u00e3o Paulo (USP\u2010RP)  Ribeir\u00e3o Preto Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celeste","family":"Montecchiani","sequence":"additional","affiliation":[{"name":"Laboratorio di Neurogenetica, Centro Europeo di Ricerca sul Cervello (CERC)\u2013Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Santa Lucia  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Orlacchio","sequence":"additional","affiliation":[{"name":"Laboratorio di Neurogenetica, Centro Europeo di Ricerca sul Cervello (CERC)\u2013Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Santa Lucia  Rome Italy"},{"name":"Dipartimento di Scienze Chirurgiche e Biomediche Universit\u00e0 di Perugia  Perugia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose Luiz","family":"Pedroso","sequence":"additional","affiliation":[{"name":"Department of Neurology Federal University of S\u00e3o Paulo (UNIFESP)  S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orlando Graziani Povoas","family":"Barsottini","sequence":"additional","affiliation":[{"name":"Department of Neurology Federal University of S\u00e3o Paulo (UNIFESP)  S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celso Dar\u00edo","family":"Ramos","sequence":"additional","affiliation":[{"name":"Division of Nuclear Medicine, Department of Radiology University of Campinas (UNICAMP)  Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00cdscia","family":"Lopes\u2010Cendes","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics University of Campinas (UNICAMP) Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2534-4160","authenticated-orcid":false,"given":"Joseph H.","family":"Friedman","sequence":"additional","affiliation":[{"name":"Department of Neurology Butler Hospital and Warren Alpert Medical School of Brown University  Providence Rhode Island USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e1rbara Juarez","family":"Amorim","sequence":"additional","affiliation":[{"name":"Division of Nuclear Medicine, Department of Radiology University of Campinas (UNICAMP)  Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Marcondes Cavalcante","family":"Fran\u00e7a","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Campinas (UNICAMP) Campinas Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,10,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1980"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aww111"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1552-6569.2008.00327.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21310"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31826d5fb0"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10592"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12149-015-1042-y"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-009-0083-3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23552"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000228242.53336.90"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1590\/0004-282x20160047"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1532-5415.2005.53221.x"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/gps.3787"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/gps.1610"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1590\/s1980-57642008dn10200015"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22340"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2011-300129"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2018.05.031"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000294327.66106.3d"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2011.7"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20945"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2015.10.006"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20054"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awt103"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26327"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12149-017-1166-3"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s007020050025"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.27491","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.27491","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T12:16:29Z","timestamp":1760962589000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.27491"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10]]},"references-count":27,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2018,10]]}},"alternative-id":["10.1002\/mds.27491"],"URL":"https:\/\/doi.org\/10.1002\/mds.27491","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10]]},"assertion":[{"value":"2018-03-05","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-07-19","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-10-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}