{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T14:25:57Z","timestamp":1791901557168,"version":"4.3.4"},"reference-count":111,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2006,5,22]],"date-time":"2006-05-22T00:00:00Z","timestamp":1148256000000},"content-version":"vor","delay-in-days":21,"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":[[2006,5]]},"abstract":"<jats:p>To review the literature on primary dystonia and dystonia plus and to provide evidence\u2010based recommendations. Primary dystonia and dystonia plus are chronic and often disabling conditions with a widespread spectrum mainly in young people. Computerized MEDLINE and EMBASE literature reviews (1966\u20131967 February 2005) were conducted. The Cochrane Library was searched for relevant citations. Diagnosis and classification of dystonia are highly relevant for providing appropriate management and prognostic information, and genetic counselling. Expert observation is suggested. DYT\u20101 gene testing in conjunction with genetic counselling is recommended for patients with primary dystonia with onset before age 30\u2003years and in those with an affected relative with early onset. Positive genetic testing for dystonia (e.g. DYT\u20101) is not sufficient to make diagnosis of dystonia. Individuals with myoclonus should be tested for the epsilon\u2010sarcoglycan gene (DYT\u201011). A levodopa trial is warranted in every patient with early onset dystonia without an alternative diagnosis. Brain imaging is not routinely required when there is a confident diagnosis of primary dystonia in adult patients, whereas it is necessary in the paediatric population. Botulinum toxin (BoNT) type A (or type B if there is resistance to type A) can be regarded as first line treatment for primary cranial (excluding oromandibular) or cervical dystonia and can be effective in writing dystonia. Actual evidence is lacking on direct comparison of the clinical efficacy and safety of BoNT\u2010A vs. BoNT\u2010B. Pallidal deep brain stimulation (DBS) is considered a good option, particularly for generalized or cervical dystonia, after medication or BoNT have failed to provide adequate improvement. Selective peripheral denervation is a safe procedure that is indicated exclusively in cervical dystonia. Intrathecal baclofen can be indicated in patients where secondary dystonia is combined with spasticity. The absolute and comparative efficacy and tolerability of drugs in dystonia, including anticholinergic and antidopaminergic drugs, is poorly documented and no evidence\u2010based recommendations can be made to guide prescribing.<\/jats:p>","DOI":"10.1111\/j.1468-1331.2006.01537.x","type":"journal-article","created":{"date-parts":[[2006,5,22]],"date-time":"2006-05-22T14:38:10Z","timestamp":1148308690000},"page":"433-444","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":228,"title":["A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS\/MDS\u2010ES Task Force"],"prefix":"10.1111","volume":"13","author":[{"given":"A.","family":"Albanese","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. P.","family":"Barnes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K. P.","family":"Bhatia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Fernandez\u2010Alvarez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Filippini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Gasser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. K.","family":"Krauss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Newton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Rektor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Savoiardo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Valls\u2010Sol\u00e8","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,5,22]]},"reference":[{"key":"e_1_2_36_2_2","first-page":"332","volume-title":"Movement Disorders 2","author":"Fahn S","year":"1987"},{"key":"e_1_2_36_3_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.50.5_Suppl_5.S1"},{"key":"e_1_2_36_4_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.74.3.348"},{"key":"e_1_2_36_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(04)00907-X"},{"key":"e_1_2_36_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2004.00867.x"},{"key":"e_1_2_36_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870131303"},{"key":"e_1_2_36_8_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.5.1213"},{"key":"e_1_2_36_9_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.111.1.e89"},{"key":"e_1_2_36_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-003-0236-8"},{"key":"e_1_2_36_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002826-200009000-00002"},{"key":"e_1_2_36_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870130329"},{"key":"e_1_2_36_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(98)00519-9"},{"key":"e_1_2_36_14_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2003.017632"},{"key":"e_1_2_36_15_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.68.2.186"},{"key":"e_1_2_36_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(89)90188-8"},{"key":"e_1_2_36_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(02)00137-0"},{"key":"e_1_2_36_18_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000035630.12515.E0"},{"key":"e_1_2_36_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10236"},{"key":"e_1_2_36_20_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.12.2335"},{"key":"e_1_2_36_21_2","doi-asserted-by":"publisher","DOI":"10.1089\/gte.1999.3.323"},{"key":"e_1_2_36_22_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.54.9.1746"},{"key":"e_1_2_36_23_2","first-page":"215","article-title":"Hereditary progressive dystonia with marked diurnal fluctuation","volume":"14","author":"Segawa M","year":"1976","journal-title":"Advances in Neurology"},{"key":"e_1_2_36_24_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2002-23590"},{"key":"e_1_2_36_25_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.6.1112"},{"key":"e_1_2_36_26_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.66.1.86"},{"key":"e_1_2_36_27_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000048205.18445.98"},{"key":"e_1_2_36_28_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.48.5.1290"},{"key":"e_1_2_36_29_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005532610051"},{"key":"e_1_2_36_30_2","first-page":"113","article-title":"Inherited myoclonus\u2010dystonia","volume":"94","author":"Asmus F","year":"2004","journal-title":"Advances in Neurology"},{"key":"e_1_2_36_31_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.9.1213"},{"key":"e_1_2_36_32_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000151979.68010.9B"},{"key":"e_1_2_36_33_2","first-page":"1314","article-title":"A major locus for myoclonus\u2010dystonia maps to chromosome 7q in eight families","volume":"67","author":"Klein C","year":"2000","journal-title":"American Journal of Human Genetics"},{"key":"e_1_2_36_34_2","doi-asserted-by":"publisher","DOI":"10.1007\/s100480100111"},{"key":"e_1_2_36_35_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10325"},{"key":"e_1_2_36_36_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.12.2596"},{"key":"e_1_2_36_37_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410380405"},{"key":"e_1_2_36_38_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2001-13121"},{"key":"e_1_2_36_39_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.61.7.1025"},{"key":"e_1_2_36_40_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh330"},{"key":"e_1_2_36_41_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.62.4.597"},{"key":"e_1_2_36_42_2","first-page":"1233","article-title":"American Society of Human Genetics Board of Directors, American College of Medical Genetics Board of Directors","volume":"57","author":"Points to consider: ethical, legal, and psychosocial implications of genetic testing in children and adolescents","year":"1995","journal-title":"American Journal of Human Genetics"},{"key":"e_1_2_36_43_2","first-page":"851","volume-title":"Motor Control Mechanisms in Health and Disease","author":"Rothwell JC","year":"1983"},{"key":"e_1_2_36_44_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.48.8.782"},{"key":"e_1_2_36_45_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.38.7.1005"},{"key":"e_1_2_36_46_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.52.9.1043"},{"key":"e_1_2_36_47_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00839204"},{"key":"e_1_2_36_48_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/108.3.593"},{"key":"e_1_2_36_49_2","first-page":"133","article-title":"Reciprocal inhibition between muscles of the human forearm in normal subjects and in patients with idiopathic torsion dystonia","volume":"50","author":"Rothwell JC","year":"1988","journal-title":"Advances in Neurology"},{"key":"e_1_2_36_50_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870030108"},{"key":"e_1_2_36_51_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/112.3.681"},{"key":"e_1_2_36_52_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.39.1.85"},{"key":"e_1_2_36_53_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.59.5.493"},{"key":"e_1_2_36_54_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.5.1371"},{"key":"e_1_2_36_55_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.49.4.1054"},{"key":"e_1_2_36_56_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.44.5.846"},{"key":"e_1_2_36_57_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8257(200101)16:1<23::AID-MDS1003>3.0.CO;2-2"},{"key":"e_1_2_36_58_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000063306.67984.8C"},{"key":"e_1_2_36_59_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004150170263"},{"key":"e_1_2_36_60_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf083"},{"key":"e_1_2_36_61_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10416"},{"key":"e_1_2_36_62_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000094240.93745.83"},{"key":"e_1_2_36_63_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20113"},{"key":"e_1_2_36_64_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.5.673"},{"key":"e_1_2_36_65_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20442"},{"key":"e_1_2_36_66_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10592"},{"key":"e_1_2_36_67_2","first-page":"265","article-title":"Magnetic resonance imaging of dystonic states","volume":"50","author":"Rutledge JN","year":"1988","journal-title":"Advances in Neurology"},{"key":"e_1_2_36_68_2","doi-asserted-by":"publisher","DOI":"10.1177\/1073858402239593"},{"key":"e_1_2_36_69_2","first-page":"1","article-title":"Clinical use of botulinum toxin","volume":"8","author":"The National Institute of Health Consensus Development","year":"1990","journal-title":"NIH Consensus Statement"},{"key":"e_1_2_36_70_2","first-page":"CD003633","article-title":"Botulinum toxin type A therapy for cervical dystonia","volume":"1","author":"Costa J","year":"2005","journal-title":"Cochrane Database System Review"},{"key":"e_1_2_36_71_2","first-page":"CD004315","article-title":"Botulinum toxin type B for cervical dystonia","volume":"1","author":"Costa J","year":"2005","journal-title":"Cochrane Database System Review"},{"key":"e_1_2_36_72_2","doi-asserted-by":"publisher","DOI":"10.2165\/00003495-200262040-00011"},{"key":"e_1_2_36_73_2","first-page":"CD004314","article-title":"Botulinum toxin type A versus botulinum toxin type B for cervical dystonia","volume":"1","author":"Costa J","year":"2005","journal-title":"Cochrane Database Systems Review"},{"key":"e_1_2_36_74_2","first-page":"CD004312","article-title":"Botulinum toxin type A versus anticholinergics for cervical dystonia","volume":"1","author":"Costa J","year":"2005","journal-title":"Cochrane Database Systems Review"},{"key":"e_1_2_36_75_2","first-page":"CD004900","article-title":"Botulinum toxin type A therapy for blepharospasm","volume":"1","author":"Costa J","year":"2005","journal-title":"Cochrane Database Systems Review"},{"key":"e_1_2_36_76_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(89)32989-7"},{"key":"e_1_2_36_77_2","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.CD004327.pub2"},{"key":"e_1_2_36_78_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2004.00845.x"},{"key":"e_1_2_36_79_2","doi-asserted-by":"publisher","DOI":"10.1053\/apmr.2001.21843"},{"key":"e_1_2_36_80_2","first-page":"189","article-title":"Double\u2010blind evaluation of trihexyphenidyl in dystonia","volume":"37","author":"Burke RE","year":"1983","journal-title":"Advances in Neurology"},{"key":"e_1_2_36_81_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.36.2.160"},{"key":"e_1_2_36_82_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.34.2.215"},{"key":"e_1_2_36_83_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100044139"},{"key":"e_1_2_36_84_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.36.1.98"},{"key":"e_1_2_36_85_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.50.11.1522"},{"key":"e_1_2_36_86_2","first-page":"561","article-title":"Dopamine agonists and antagonists in the treatment of idiopathic dystonia","volume":"50","author":"Lang AE","year":"1988","journal-title":"Advances in Neurology"},{"key":"e_1_2_36_87_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410110108"},{"key":"e_1_2_36_88_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.48.2.358"},{"key":"e_1_2_36_89_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.58.6.905"},{"key":"e_1_2_36_90_2","doi-asserted-by":"publisher","DOI":"10.1590\/S0004-282X1995000200022"},{"key":"e_1_2_36_91_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410350405"},{"key":"e_1_2_36_92_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1353-8020(00)00084-5"},{"key":"e_1_2_36_93_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.1280"},{"key":"e_1_2_36_94_2","doi-asserted-by":"publisher","DOI":"10.1097\/00004691-200401000-00004"},{"key":"e_1_2_36_95_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa042187"},{"key":"e_1_2_36_96_2","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2004.101.2.0189"},{"key":"e_1_2_36_97_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10380"},{"key":"e_1_2_36_98_2","doi-asserted-by":"publisher","DOI":"10.1227\/01.NEU.0000143331.86101.5E"},{"key":"e_1_2_36_99_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100003401"},{"key":"e_1_2_36_100_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)02410-7"},{"key":"e_1_2_36_101_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(99)80022-1"},{"key":"e_1_2_36_102_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.1092"},{"key":"e_1_2_36_103_2","volume-title":"Selective Peripheral Denervation of Cervical Dystonia","author":"The National Institute for Clinical Excellence","year":"2004"},{"key":"e_1_2_36_104_2","doi-asserted-by":"publisher","DOI":"10.1016\/0090-3019(93)90118-K"},{"key":"e_1_2_36_105_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0012162201001190"},{"key":"e_1_2_36_106_2","first-page":"299","volume-title":"Surgery for Parkinson's Disease and Movement Disorders","author":"Ondo WG","year":"2001"},{"key":"e_1_2_36_107_2","doi-asserted-by":"publisher","DOI":"10.1159\/000076654"},{"key":"e_1_2_36_108_2","doi-asserted-by":"publisher","DOI":"10.1097\/00006123-200104000-00025"},{"key":"e_1_2_36_109_2","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1093\/neurosurgery\/17.CN_suppl_1.28","article-title":"Schiffer S: Spasmodic torticollis; results after cervical rhizotomy in 80 cases","volume":"17","author":"Hamby WB","year":"1970","journal-title":"Clinical Neurosurgery"},{"key":"e_1_2_36_110_2","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1993.78.1.0046"},{"key":"e_1_2_36_111_2","doi-asserted-by":"publisher","DOI":"10.1016\/0090-3019(86)90285-5"},{"key":"e_1_2_36_112_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01428497"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1468-1331.2006.01537.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1468-1331.2006.01537.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T12:52:56Z","timestamp":1696942376000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1468-1331.2006.01537.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,5]]},"references-count":111,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2006,5]]}},"alternative-id":["10.1111\/j.1468-1331.2006.01537.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1468-1331.2006.01537.x","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,5]]},"assertion":[{"value":"2006-05-22","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}