| Title: |
Primary Lateral Sclerosis:Implications for Diagnostic Criteria From a Natural History Study in the Netherlands |
| Authors: |
de Vries, Bálint S; de Boer, Eva Maria Johanna; Brugman,Frans; Van Damme,Philip; Veldink, Jan Herman; van Es, Michael A; van den Berg, Leonard H; Projectafdeling ALS; Opleiding Neurologie; Neurologen; Brain; Genetic Risks; Neuromuscular Disorders |
| Publication Year: |
2025 |
| Subject Terms: |
Adult; Aged; Amyotrophic Lateral Sclerosis/diagnosis; Cohort Studies; Disease Progression; Female; Follow-Up Studies; Humans; Male; Middle Aged; Motor Neuron Disease/diagnosis; Netherlands/epidemiology; Journal Article; Validation Studies |
| Description: |
Background and ObjectivesPrimary lateral sclerosis (PLS) is a rare disease characterized by upper motor neuron (UMN) degeneration. We aimed to elucidate the natural history in patients with UMN syndromes suggestive of PLS and validate the most recent diagnostic (consensus) criteria.MethodsA validation study of a long-term follow-up cohort was conducted, including adults with UMN syndromes and disease durations ≥6 months. Patients were assessed at baseline (T1), at 3 years (T2), and when possible after 13 years (T3). Diagnostic categorization followed the 2020 PLS consensus criteria. Main outcomes included diagnostic classification at follow-up and survival.ResultsThe study comprised 86 patients (34 women [40%], mean age 58.9 ± 10.1 years), of whom 43 met the PLS consensus criteria at baseline (6 probable, 37 definite). Eight patients had a disease duration |
| Document Type: |
article in journal/newspaper |
| File Description: |
application/pdf |
| Language: |
English |
| ISSN: |
0028-3878 |
| Relation: |
https://dspace.library.uu.nl/handle/1874/466197 |
| Availability: |
https://dspace.library.uu.nl/handle/1874/466197 |
| Rights: |
info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.DD0682C3 |
| Database: |
BASE |