Primary lateral sclerosis: consensus diagnostic criteria
Delegates of the 2nd International PLS Conference
(2020) Journal of neurology, neurosurgery, and psychiatry, volume 91, issue 4, pp. 373 - 377
(Article)
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
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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.
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Keywords: Amyotrophic Lateral Sclerosis/diagnosis, Consensus, Delayed Diagnosis, Diagnosis, Differential, Humans, Motor Neuron Disease/diagnosis, Motor Neurons/pathology, Clinical Neurology, Psychiatry and Mental health, Surgery, Journal Article, Research Support, Non-U.S. Gov't
ISSN: 0022-3050
Publisher: BMJ Publishing Group
Note: Funding Information: MRT is supported by the Motor Neurone Disease Association. MCK was supported by the National Health and Medical Research Council of Australia Program Grant (#1132524), Partnership Project (1153439) and Practitioner Fellowship (1156093). VS was supported by the Italian Ministry of Health, AriSLA (Fondazione Italiana di Ricerca per la SLA), and E-R are Joint Transnational Call. ZS received funding from Cytokinetics, Biohaven & Biogen. HM received funding from CDC, MDA, SPF, Cytokinetics and Tsumura. Funding Information: Funding MrT is supported by the Motor neurone Disease association. McK was supported by the national health and Medical research council of australia Program grant (#1132524), Partnership Project (1153439) and Practitioner Fellowship (1156093). Vs was supported by the italian Ministry of health, arisla (Fondazione italiana di ricerca per la sla), and e-rare Joint Transnational call. Zs received funding from cytokinetics, Biohaven & Biogen. hM received funding from cDc, MDa, sPF, cytokinetics and Tsumura. Publisher Copyright: © 2020 Author(s).
(Peer reviewed)