{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T11:21:42Z","timestamp":1793186502718,"version":"4.5.0"},"reference-count":42,"publisher":"BMJ","issue":"4","license":[{"start":{"date-parts":[[2020,2,6]],"date-time":"2020-02-06T00:00:00Z","timestamp":1580947200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000406","name":"Motor Neurone Disease Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000406","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["1132524, 1153439, 1156093"],"award-info":[{"award-number":["1132524, 1153439, 1156093"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2020,4]]},"abstract":"<jats:p>Primary lateral sclerosis (PLS) is a neurodegenerative disorder of the adult motor system. Characterised by a slowly progressive upper motor neuron syndrome, the diagnosis is clinical, after exclusion of structural, neurodegenerative and metabolic mimics. Differentiation of PLS from upper motor neuron-predominant forms of amyotrophic lateral sclerosis remains a significant challenge in the early symptomatic phase of both disorders, with ongoing debate as to whether they form a clinical and histopathological continuum. Current diagnostic criteria for PLS may be a barrier to therapeutic development, requiring long delays between symptom onset and formal diagnosis. While new technologies sensitive to both upper and lower motor neuron involvement may ultimately resolve controversies in the diagnosis of PLS, we present updated consensus diagnostic criteria with the aim of reducing diagnostic delay, optimising therapeutic trial design and catalysing the development of disease-modifying therapy.<\/jats:p>","DOI":"10.1136\/jnnp-2019-322541","type":"journal-article","created":{"date-parts":[[2020,2,6]],"date-time":"2020-02-06T22:15:38Z","timestamp":1581027338000},"page":"373-377","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":231,"title":["Primary lateral sclerosis: consensus diagnostic criteria"],"prefix":"10.1136","volume":"91","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0267-3180","authenticated-orcid":false,"given":"Martin R","family":"Turner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J","family":"Barohn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Corcia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John K","family":"Fink","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew B","family":"Harms","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew C","family":"Kiernan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Ravits","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Silani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zachary","family":"Simmons","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0790-5315","authenticated-orcid":false,"given":"Jeffrey","family":"Statland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonard H","family":"van den Berg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Delegates of the 2nd International PLS Conference","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Mitsumoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2020,2,6]]},"reference":[{"key":"key-10.1136\/jnnp-2019-322541-1","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1097\/00005053-194510000-00002","article-title":"Primary lateral sclerosis: a distinct clinical entity","volume":"102","author":"Stark","year":"1945","journal-title":"J Nerv Ment Dis"},{"key":"key-10.1136\/jnnp-2019-322541-2","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1136\/jnnp.2010.235952","article-title":"Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study","volume":"82","author":"Chi\u00f2","year":"2011","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2019-322541-3","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1002\/mus.23496","article-title":"Clinical evolution of pure upper motor neuron disease\/dysfunction (PUMMD)","volume":"47","author":"D'Amico","year":"2013","journal-title":"Muscle Nerve"},{"key":"key-10.1136\/jnnp-2019-322541-4","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1093\/brain\/115.2.495","article-title":"Primary lateral sclerosis. clinical features, neuropathology and diagnostic criteria","volume":"115","author":"Pringle","year":"1992","journal-title":"Brain"},{"key":"key-10.1136\/jnnp-2019-322541-5","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1212\/01.wnl.0000200962.94777.71","article-title":"The natural history of primary lateral sclerosis","volume":"66","author":"Gordon","year":"2006","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2019-322541-6","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1007\/s00401-003-0687-0","article-title":"Primary lateral sclerosis: a rare upper-motor-predominant form of amyotrophic lateral sclerosis often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions? 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