TY  - JOUR
N1  - © 2025 Springer Nature. This article is licensed under a Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/).
IS  - 3
TI  - Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score
EP  - 8
Y1  - 2025///
AV  - public
VL  - 272
JF  - Journal of Neurology
KW  - Motor neuron disease
KW  -  Diagnosis
KW  -  Upper motor neuron
KW  -  Reliability
A1  - Jacobsen, Anna B
A1  - Fanella, Gaia
A1  - de Carvalho, Mamede
A1  - Koltzenburg, Martin
A1  - Oliveira Santos, Miguel
A1  - Cengiz, Bulent
A1  - Blicher, Jakob
A1  - Obal, Izabella
A1  - Heintzelmann, Mia B
A1  - Nix, Wilfred
A1  - Camdessanche, Jean-Philippe
A1  - Fuglsang-Frederiksen, Anders
A1  - Tankisi, Hatice
ID  - discovery10205922
N2  - 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.
UR  - https://doi.org/10.1007/s00415-025-12895-7
PB  - SPRINGER HEIDELBERG
ER  -