Primary lateral sclerosis: consensus diagnostic criteria. Issue 4 (6th February 2020)

• Record Type: Journal Article
• Authors: Turner, Martin R; Barohn, Richard J; Corcia, Philippe; Fink, John K; Harms, Matthew B; Kiernan, Matthew C; Ravits, John; Silani, Vincenzo; Simmons, Zachary; Statland, Jeffrey; van den Berg, Leonard H; Mitsumoto, Hiroshi
• Other Names: author non-byline.; Ajroud-Driss Senda author non-byline.; Andrews Jinsy A author non-byline.; Babu Suma author non-byline.; Bede Peter author non-byline.; Benatar Michael author non-byline.; Chew Sheena author non-byline.; Conwit Robin author non-byline.; Cudkowicz Merit author non-byline.; Carvalho Mamede de author non-byline.; Drory Vivian E author non-byline.; Elman Lauren author non-byline.; M Fernandes J Americo author non-byline.; Ferrey Dominic author non-byline.; Finegan Eoin author non-byline.; Floeter Mary Kay author non-byline.; Fournier Christina N author non-byline.; Govindarajan Raghav author non-byline.; Granit Volkan author non-byline.; Hardiman Orla author non-byline.; Hayat Ghazala author non-byline.; Heiman-Patterson Terry D author non-byline.; Hübers Annemarie author non-byline.; Huey Edward D author non-byline.; Jawdat Omar author non-byline.; Kano Osamu author non-byline.; Kisanuki Yaz Y author non-byline.; Mackenzie Ian author non-byline.; Marren David author non-byline.; Morita Mitsuya author non-byline.; Murphy Jennifer author non-byline.; Nations Sharon author non-byline.; Oskarsson Björn author non-byline.; Paganoni Sabrina author non-byline.; Pioro Erik P author non-byline.; Pullman Seth L author non-byline.; Rezania Kourosh author non-byline.; Rouleau Guy author non-byline.; Scelsa Stephen author non-byline.; Siddique Nailah author non-byline.; Solano Juan author non-byline.; Blitterswijk Marka van author non-byline.; Walk David author non-byline.; Wymer James author non-byline.; Zhao Cuiping author non-byline.; Lange Dale author non-byline.;
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 91:Issue 4(2020)
• Page Start: 373
• Page End: 377
• Publication Date: 2020-02-06
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2019-322541
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : 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.