{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T17:14:49Z","timestamp":1788023689167,"version":"build-2784847793"},"reference-count":25,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,11,28]],"date-time":"2019-11-28T00:00:00Z","timestamp":1574899200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:p>The aim was to review the existing reports on cognitive and behavioural symptoms in monogenic forms of Parkinson\u2019s disease (PD) and to identify recurring patterns of clinical manifestations in those with specific mutations. A systematic literature search was conducted to retrieve observational studies of monogenic PD. Data pertaining to cognitive and psychiatric manifestations were extracted using standardized templates. The PRISMA guidelines were followed. Of the 1889 citations retrieved, 95 studies on PD\u2010related gene mutations were included: 35 in <jats:italic>SNCA<\/jats:italic>, 35 in <jats:italic>LRRK2<\/jats:italic>, four in <jats:italic>VPS35<\/jats:italic>, 10 in <jats:italic>Parkin<\/jats:italic>, three in <jats:italic>DJ1<\/jats:italic> and eight in <jats:italic>PINK1<\/jats:italic>. Nineteen studies (20%) provided adequate data from comprehensive cognitive assessment and 31 studies (32.6%) outlined psychiatric manifestations through the use of neuropsychiatric scales. Cognitive impairment was reported in all monogenic PD forms with variable rates (58.8% <jats:italic>PINK1<\/jats:italic>, 53.9% <jats:italic>SNCA<\/jats:italic>, 50% <jats:italic>DJ1<\/jats:italic>, 29.2% <jats:italic>VPS35<\/jats:italic>, 15.7% <jats:italic>LRRK2<\/jats:italic> and 7.4% <jats:italic>Parkin<\/jats:italic>). In this regard, executive functions and attention were the domains most affected. With respect to psychiatric symptoms, depression was the most frequent symptom, occurring in 37.5% of <jats:italic>PINK1<\/jats:italic> cases and 41.7% of <jats:italic>VPS35<\/jats:italic> and <jats:italic>LRRK2<\/jats:italic> cases. Co\u2010occurrence of cognitive decline with visual hallucinations was evidenced. Widespread accumulation of Lewy bodies, distinctive of <jats:italic>SNCA, PINK1<\/jats:italic> and <jats:italic>DJ1 <\/jats:italic>mutations, results in higher rates of cognitive impairment. Similarly, a higher degree of visual hallucinations is observed in <jats:italic>SNCA<\/jats:italic> mutations, probably owing to the more widespread accumulation. The lower rates of \u03b1\u2010synuclein pathology in <jats:italic>LRRK2<\/jats:italic> and <jats:italic>Parkin<\/jats:italic> may underpin the more benign disease course in these patients.<\/jats:p>","DOI":"10.1111\/ene.14115","type":"journal-article","created":{"date-parts":[[2019,11,5]],"date-time":"2019-11-05T09:50:37Z","timestamp":1572947437000},"page":"229-234","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":50,"title":["Cognitive and psychiatric symptoms in genetically determined Parkinson\u2019s disease: a systematic review"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0325-8667","authenticated-orcid":false,"given":"R.","family":"Piredda","sequence":"first","affiliation":[{"name":"CINAC\u2010HM Puerta del Sur CEU\u2010San Pablo University M\u00f3stoles, Madrid Spain"},{"name":"Department of Neurology IRCCS Istituto Clinico Humanitas Rozzano Milano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7516-9594","authenticated-orcid":false,"given":"P.","family":"Desmarais","sequence":"additional","affiliation":[{"name":"Cognitive and Movement Disorders Clinic Sunnybrook Health Sciences Centre Toronto Canada"},{"name":"L.C. Campbell Cognitive Neurology Research Unit Sunnybrook Health Sciences Centre Toronto Canada"},{"name":"Hurvitz Brain Sciences Program Sunnybrook Research Institute University of Toronto Toronto Canada"},{"name":"Division of Neurology Department of Medicine University of Toronto Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6244-2096","authenticated-orcid":false,"given":"M.","family":"Masellis","sequence":"additional","affiliation":[{"name":"Cognitive and Movement Disorders Clinic Sunnybrook Health Sciences Centre Toronto Canada"},{"name":"L.C. Campbell Cognitive Neurology Research Unit Sunnybrook Health Sciences Centre Toronto Canada"},{"name":"Hurvitz Brain Sciences Program Sunnybrook Research Institute University of Toronto Toronto Canada"},{"name":"Division of Neurology Department of Medicine University of Toronto Toronto Canada"},{"name":"Institute of Medical Science University of Toronto Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7411-8106","authenticated-orcid":false,"given":"C.","family":"Gasca\u2010Salas","sequence":"additional","affiliation":[{"name":"CINAC\u2010HM Puerta del Sur CEU\u2010San Pablo University M\u00f3stoles, Madrid Spain"},{"name":"CIBERNED Institute Carlos III Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,11,28]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.276.5321.2045"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-444-63233-3.00014-2"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/bs.irn.2017.05.030"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.b.32549"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2015.06.005"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2011.10.009"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2008.06.008"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.17925\/ENR.2009.04.01.40"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000142258.29304.FE"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10846"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21966"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awq051"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl114"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10675"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.51.3.890"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8257(200009)15:5<884::AID-MDS1019>3.0.CO;2-8"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20791"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000240220.33950.0c"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awy072"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awv104"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21803"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2011-300980"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181c34b47"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21956"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0197-4580(02)00065-9"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.14115","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.14115","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.14115","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,8]],"date-time":"2023-09-08T08:09:38Z","timestamp":1694160578000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.14115"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,28]]},"references-count":25,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2020,2]]}},"alternative-id":["10.1111\/ene.14115"],"URL":"https:\/\/doi.org\/10.1111\/ene.14115","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,11,28]]},"assertion":[{"value":"2018-12-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-11-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-11-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}