Journal article
Primary lateral sclerosis: consensus diagnostic criteria
- 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.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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(Preview, Version of record, pdf, 357.1KB, Terms of use)
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- Publisher copy:
- 10.1136/jnnp-2019-322541
Authors
- Publisher:
- BMJ Publishing Group
- Journal:
- Journal of Neurology, Neurosurgery, and Psychiatry More from this journal
- Volume:
- 91
- Issue:
- 4
- Pages:
- 373-377
- Place of publication:
- England
- Publication date:
- 2020-02-06
- Acceptance date:
- 2019-12-27
- DOI:
- EISSN:
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1468-330X
- ISSN:
-
0022-3050
- Pmid:
-
32029539
- Language:
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English
- Keywords:
- Pubs id:
-
1087211
- Local pid:
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pubs:1087211
- Deposit date:
-
2020-03-23
- ARK identifier:
Terms of use
- Copyright holder:
- Turner et al.
- Copyright date:
- 2020
- Rights statement:
- © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits others to copy, redistribute, remix, transform and build upon this work for any purpose, provided the original work is properly cited, a link to the licence is given, and indication of whether changes were made. See: https://creativecommons.org/licenses/by/4.0/.
- Licence:
- CC Attribution (CC BY)
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