{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T11:24:03Z","timestamp":1783077843528,"version":"3.54.6"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2015,5,21]],"date-time":"2015-05-21T00:00:00Z","timestamp":1432166400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2015,5,21]],"date-time":"2015-05-21T00:00:00Z","timestamp":1432166400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["npj Parkinson's Disease"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background\/Objectives:<\/jats:title>\n                <jats:p>The clinical diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remains challenging due to heterogeneity of the diseases.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>AIMS:<\/jats:title>\n                <jats:p>Here we compared the clinical features of PSP and MSA to gain insight into their diagnosis and prognosis, particularly the prognostic value of down-gaze palsy latency in PSP progression.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods:<\/jats:title>\n                <jats:p>We reviewed clinical features of pathologically confirmed 10 PSP and 13 MSA patients, incidentally matched in age-at-onset, gender, and disease duration, followed at Columbia University Medical Center during 1994\u20132009.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results:<\/jats:title>\n                <jats:p>The final antemortem diagnosis was incorrect in 30% of PSP (all lacking down-gaze palsy) and 23% of MSA patients. Falls in the first year of the disease, pyramidal involvement and freezing of gait during the course were similar between PSP and MSA. Ataxia and apraxia were in 50% of the PSP patients. Parkinsonism responsive to levodopa treatment was in 30% of the PSP (all with resting tremor) and 50% of the MSA patients. Dysautonomia in MSA could occur as early as 3 years preceding the motor symptoms, with 46% within the first year of the motor onset, but 15% did not have dysautonomia in life. The latency of down-gaze palsy and urogenital dysfunction and MMSE scores at first visit in PSP, and the latency of falls and wheelchair confinement in MSA were all associated with the disease progression.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions:<\/jats:title>\n                <jats:p>We confirmed most of the previously published characterizations, and identified for the first time the prognostic value of down-gaze palsy latency in PSP progression.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1038\/npjparkd.2015.7","type":"journal-article","created":{"date-parts":[[2015,5,21]],"date-time":"2015-05-21T14:59:57Z","timestamp":1432220397000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center"],"prefix":"10.1038","volume":"1","author":[{"given":"Tao","family":"Xie","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Un J","family":"Kang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheng-Han","family":"Kuo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markos","family":"Poulopoulos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Greene","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanley","family":"Fahn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2015,5,21]]},"reference":[{"key":"BFnpjparkd20157_CR1","doi-asserted-by":"publisher","first-page":"922","DOI":"10.1212\/WNL.46.4.922","volume":"46","author":"I Litvan","year":"1996","unstructured":"Litvan I, Agid Y, Jankovic J, Goetz C, Brandel JP, Lai EC et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology 1996; 46: 922\u2013930.","journal-title":"Neurology"},{"key":"BFnpjparkd20157_CR2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1212\/WNL.47.1.1","volume":"47","author":"I Litvan","year":"1996","unstructured":"Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC et al. Clinical research criteria for the progressive supranuclear palsy (Steele-Ricardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 1996; 47: 1\u20139.","journal-title":"Neurology"},{"key":"BFnpjparkd20157_CR3","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1136\/jnnp.60.6.615","volume":"60","author":"I Litvan","year":"1996","unstructured":"Litvan I, Mangone CA, Mckee A, Verny M, Parsa A, Jellinger K et al. Natural history of progressive supranuclear palsy (Steele-Ricardson-Olszewski syndrome) and clinical predictor of the survival: a clinicoathological study. J Neurol Neurosurg Psychiatry 1996; 60: 615\u2013620.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"BFnpjparkd20157_CR4","doi-asserted-by":"publisher","first-page":"167","DOI":"10.1136\/jnnp.58.2.167","volume":"58","author":"SJ Collins","year":"1995","unstructured":"Collins SJ, Ahlskog JE, Parisi JE, Maraganore DM . Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria. J Neurol Neurosurg Psychiatry 1995; 58: 167\u2013173.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"BFnpjparkd20157_CR5","doi-asserted-by":"publisher","first-page":"1255","DOI":"10.1002\/mds.10211","volume":"17","author":"S Birdi","year":"2002","unstructured":"Birdi S, Rajput AH, Frenton M, Donat JR, Rozdilsky B, Robinson C et al. Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases. Mov Disord 2002; 17: 1255\u20131264.","journal-title":"Mov Disord"},{"key":"BFnpjparkd20157_CR6","doi-asserted-by":"publisher","first-page":"759","DOI":"10.1093\/brain\/118.3.759","volume":"118","author":"SE Daniel","year":"1995","unstructured":"Daniel SE, de Bruin VM, Lees AJ . The clinical and pathological spectrum of Steele-Richardson-Olszeweski syndrome (progressive supranuclear palsy): a reappraisal. Brain 1995; 118: 759\u2013770.","journal-title":"Brain"},{"key":"BFnpjparkd20157_CR7","doi-asserted-by":"publisher","first-page":"670","DOI":"10.1212\/01.wnl.0000324625.00404.15","volume":"71","author":"S Gilman","year":"2008","unstructured":"Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008; 71: 670\u2013676.","journal-title":"Neurology"},{"key":"BFnpjparkd20157_CR8","doi-asserted-by":"publisher","first-page":"1247","DOI":"10.1093\/brain\/awh488","volume":"128","author":"DR Williams","year":"2005","unstructured":"Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson\u2019s syndrome and PSP-parkinsonism. Brain 2005; 128: 1247\u20131258.","journal-title":"Brain"},{"key":"BFnpjparkd20157_CR9","doi-asserted-by":"publisher","first-page":"1566","DOI":"10.1093\/brain\/awm104","volume":"130","author":"D Williams","year":"2007","unstructured":"Williams D, Holton JL, Strand C, Pittman A, de Silva R, Lee AJ et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson\u2019s syndrome. Brain 2007; 130: 1566\u20131576.","journal-title":"Brain"},{"key":"BFnpjparkd20157_CR10","doi-asserted-by":"publisher","first-page":"1362","DOI":"10.1093\/brain\/awn065","volume":"131","author":"SS O\u2019Sullivan","year":"2008","unstructured":"O\u2019Sullivan SS, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain 2008; 131: 1362\u20131372.","journal-title":"Brain"},{"key":"BFnpjparkd20157_CR11","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1016\/S1474-4422(09)70042-0","volume":"8","author":"D Williams","year":"2009","unstructured":"Williams D, Lees AJ . Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 2009; 8: 270\u2013279.","journal-title":"Lancet Neurol"},{"key":"BFnpjparkd20157_CR12","doi-asserted-by":"publisher","first-page":"1149","DOI":"10.1016\/j.parkreldis.2013.07.019","volume":"19","author":"M Kanazawa","year":"2013","unstructured":"Kanazawa M, Tada M, Onodera O, Takahashi H, Nishizawa M, Shimohata T . Early clinical features of patients with progressive supranuclear palsy with predominant cerebellar ataxia. Parkinsonism Relat Disord 2013; 19: 1149\u20131151.","journal-title":"Parkinsonism Relat Disord"},{"key":"BFnpjparkd20157_CR13","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1097\/NEN.0b013e31827ae56e","volume":"72","author":"ED Louis","year":"2013","unstructured":"Louis ED, Babij R, Ma K, Cortes E, Vonsattel JP . Essential tremor followed by progressive supranuclear palsy: postmortem reports of 11patients. J Neuropathol Exp Neurol 2013; 72: 8\u201317.","journal-title":"J Neuropathol Exp Neurol"},{"key":"BFnpjparkd20157_CR14","doi-asserted-by":"publisher","first-page":"210","DOI":"10.1055\/s-0034-1381732","volume":"34","author":"EP Ramirez","year":"2014","unstructured":"Ramirez EP, Vonsattel JP . Neuropathologic changes of multiple system atrophy and diffuse Lewy body disease. Semin Neurol 2014; 34: 210\u2013216.","journal-title":"Semin Neurol"},{"key":"BFnpjparkd20157_CR15","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1212\/WNL.41.1.25","volume":"41","author":"JL Juncos","year":"1991","unstructured":"Juncos JL, Hirsch EC, Malessa S, Duyckaerts C, Hersh LB, Agid Y . Mesencephalic cholinergic nuclei in progressive supranuclear palsy. Neurology 1991; 41: 25\u201330.","journal-title":"Neurology"},{"key":"BFnpjparkd20157_CR16","doi-asserted-by":"publisher","first-page":"620","DOI":"10.1136\/jnnp.67.5.620","volume":"67","author":"GK Wenning","year":"1999","unstructured":"Wenning GK, Scherfler C, Granata R, Bosch S, Vermy M, Chaudhuri KR et al. Time course of symptomatic orthostatic hypotension and urinary incontinence in patients with postmortem confirmed parkinsonian syndromes: a clinicopathological study. J Neurol Neurosurg Psychiatry 1999; 67: 620\u2013623.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"BFnpjparkd20157_CR17","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1159\/000086754","volume":"54","author":"S Papapetropoulos","year":"2005","unstructured":"Papapetropoulos S, Gonzalez J, Mash DC . Natural history of progressive supranuclear palsy: a clinicopathologic study from a population of brain donors. Eur Neurol 2005; 54: 1\u20139.","journal-title":"Eur Neurol"},{"key":"BFnpjparkd20157_CR18","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1136\/jnnp.68.4.434","volume":"68","author":"GK Wenning","year":"2000","unstructured":"Wenning GK, Ben-Shlomo Y, Hughes A, Daniel SE, Lees A, Quinn NP . What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson\u2019s disease? J Neurol Neurosurg Psychiatry 2000; 68: 434\u2013440.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"BFnpjparkd20157_CR19","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1111\/j.1750-3639.2007.00054.x","volume":"17","author":"DW Dickson","year":"2007","unstructured":"Dickson DW, Rademakers R, Hutton ML . Progressive supranuclear palsy: pathology and genetics. Brain Pathol 2007; 17: 74\u201382.","journal-title":"Brain Pathol"},{"key":"BFnpjparkd20157_CR20","doi-asserted-by":"publisher","first-page":"1571","DOI":"10.1212\/WNL.40.10.1571","volume":"40","author":"J Rivest","year":"1990","unstructured":"Rivest J, Quinn N, Marsden CD . Dystonia in Parkinson\u2019s disease, multiple system atrophy, and progressive supranuclear palsy. Neurology 1990; 40: 1571\u20131578.","journal-title":"Neurology"}],"container-title":["npj Parkinson's Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/npjparkd20157.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/npjparkd20157","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/npjparkd20157.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,1,5]],"date-time":"2023-01-05T12:56:58Z","timestamp":1672923418000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/npjparkd20157"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,5,21]]},"references-count":20,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2015,12,17]]}},"alternative-id":["BFnpjparkd20157"],"URL":"https:\/\/doi.org\/10.1038\/npjparkd.2015.7","relation":{},"ISSN":["2373-8057"],"issn-type":[{"value":"2373-8057","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,5,21]]},"assertion":[{"value":"1 February 2015","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 April 2015","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 April 2015","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 May 2015","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no conflict of interest.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"15007"}}