eprintid: 10205922 rev_number: 9 eprint_status: archive userid: 699 dir: disk0/10/20/59/22 datestamp: 2025-03-12 13:48:16 lastmod: 2025-03-12 13:48:16 status_changed: 2025-03-12 13:48:16 type: article metadata_visibility: show sword_depositor: 699 creators_name: Jacobsen, Anna B creators_name: Fanella, Gaia creators_name: de Carvalho, Mamede creators_name: Koltzenburg, Martin creators_name: Oliveira Santos, Miguel creators_name: Cengiz, Bulent creators_name: Blicher, Jakob creators_name: Obal, Izabella creators_name: Heintzelmann, Mia B creators_name: Nix, Wilfred creators_name: Camdessanche, Jean-Philippe creators_name: Fuglsang-Frederiksen, Anders creators_name: Tankisi, Hatice title: Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score ispublished: pub divisions: UCL divisions: B02 divisions: C07 divisions: D07 divisions: F84 keywords: Motor neuron disease, Diagnosis, Upper motor neuron, Reliability note: © 2025 Springer Nature. This article is licensed under a Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/). abstract: There is a need for a consensus on a clinical scale for evaluating upper motor neuron (UMN) burden in amyotrophic lateral sclerosis (ALS) to improve consistency in clinical diagnosis, research and monitoring of disease progression. The Penn upper motor neuron score (PUMNS) is the most commonly published scale, however, the reliability of the scale has only been evaluated in a single study involving two raters. The objective of this study was to evaluate the inter-rater reliability of the PUMNS in ALS patients among multiple raters, and to discuss an updated UMN score including the signs with the highest inter-rater reliability. This study included seven ALS patients (mean age: 71 ± 11.5, six males, one female). Each patient was evaluated with the PUMNS by eight raters from different centers blinded to previous observations. The intra-class correlation coefficient (ICC) was calculated to assess the inter-rater reliability of the total PUMNS. The inter-rater reliability of the binary subscores was assessed with Gwet's AC1 coefficient. The inter-rater agreement for the total PUMNS yielded an ICC of 0.81 (95% CI 0.56;0.96). Items with the highest inter-rater reliability included Hoffman's sign, Babinski's sign, clonus and deep tendon reflexes, while the facial reflex (Gwet's AC1 -0.038 (95% CI -0.25,0.18)) and crossed adduction (0.18 (95% CI (-0.32,0.67)) had the lowest inter-rater reliability. In conclusion, PUMNS demonstrated good inter-rater reliability overall, while some of the subscores had poor inter-rater reliability. Based on this, we call for an updated UMN score to enhance diagnostic accuracy and research consistency in ALS. date: 2025 date_type: published publisher: SPRINGER HEIDELBERG official_url: https://doi.org/10.1007/s00415-025-12895-7 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 2364995 doi: 10.1007/s00415-025-12895-7 medium: Electronic pii: 10.1007/s00415-025-12895-7 lyricists_name: Koltzenburg, Martin lyricists_id: MKOLT33 actors_name: Koltzenburg, Martin actors_id: MKOLT33 actors_role: owner funding_acknowledgements: [Aarhus University Hospital]; R346-2020-1946 [Lundbeck Foundation]; R392-2022-699 [Lundbeck Foundation]; 23-2B-12533 [Aage og Johanne Louis-Hansens Fond] full_text_status: public publication: Journal of Neurology volume: 272 number: 3 article_number: 208 pages: 8 event_location: Germany citation: Jacobsen, Anna B; Fanella, Gaia; de Carvalho, Mamede; Koltzenburg, Martin; Oliveira Santos, Miguel; Cengiz, Bulent; Blicher, Jakob; ... Tankisi, Hatice; + view all <#> Jacobsen, Anna B; Fanella, Gaia; de Carvalho, Mamede; Koltzenburg, Martin; Oliveira Santos, Miguel; Cengiz, Bulent; Blicher, Jakob; Obal, Izabella; Heintzelmann, Mia B; Nix, Wilfred; Camdessanche, Jean-Philippe; Fuglsang-Frederiksen, Anders; Tankisi, Hatice; - view fewer <#> (2025) Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score. Journal of Neurology , 272 (3) , Article 208. 10.1007/s00415-025-12895-7 <https://doi.org/10.1007/s00415-025-12895-7>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10205922/3/Koltzenburg_Variability%20of%20the%20Penn%20upper%20motor%20neuron%20score%20in%20amyotrophic%20lateral%20sclerosis_VoR.pdf