Journal article
Progression and life expectancy in primary lateral sclerosis
- Abstract:
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Objectives To characterise the clinical characteristics and longitudinal outcomes in primary lateral sclerosis (PLS), including median survival from symptom onset and age at death.
Methods The authors retrospectively reviewed electronic health records of patients diagnosed with PLS referred to a specialised motor neuron disorders clinic from 2002 to 2024, analysed longitudinal Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) assessments using joint models and used Kaplan-Meier methods and life tables to calculate median survival and age at death compared with population-based values.
Results Of 52 patients, 34 (65%) were male, 41 (79%) first noted symptoms in the lower limbs and 10 (19%) in corticobulbar function. Median age of symptom onset was 53 years. The mean annual rate of functional decline was −1.92 ALSFRS-R points (95% CI −3.03 to −0.78), with equal highest rates of decline in fine and gross motor subscores. Five patients (10%) received gastrostomy and three (6%) non-invasive ventilation. Median survival from symptom onset was 23.1 years (22.7 to not reached), and median age at death was 79.5 years (77.8 to not reached) compared with a population-based reference mean of 81.9 years (81.1 to 82.8).
Discussion PLS may be commensurate with near-normal life expectancy. Significant disability arises from limb motor dysfunction, with a minority of patients requiring nutritional or respiratory support. This has important implications for counselling and trial design.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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(Preview, Version of record, pdf, 804.8KB, Terms of use)
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- Publisher copy:
- 10.1136/jnnp-2025-336037
Authors
- Funder identifier:
- https://ror.org/03x94j517
- Grant:
- MR/Y001095/1
- Publisher:
- BMJ Publishing Group
- Journal:
- Journal of Neurology, Neurosurgery and Psychiatry More from this journal
- Volume:
- 96
- Issue:
- 10
- Pages:
- 1008-1011
- Publication date:
- 2025-05-16
- Acceptance date:
- 2025-05-03
- DOI:
- EISSN:
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1468-330X
- ISSN:
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0022-3050
- Language:
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English
- Keywords:
- Pubs id:
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2125500
- Local pid:
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pubs:2125500
- Deposit date:
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2025-05-22
- ARK identifier:
Terms of use
- Copyright holder:
- Lester et al
- Copyright date:
- 2025
- Rights statement:
- © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY. Published by BMJ Group. 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.
- Licence:
- CC Attribution (CC BY)
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