TY - JOUR
T1 - Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis
T2 - need for a revised score
AU - Jacobsen, Anna B
AU - Fanella, Gaia
AU - de Carvalho, Mamede
AU - Koltzenburg, Martin
AU - Oliveira Santos, Miguel
AU - Cengiz, Bülent
AU - Blicher, Jakob
AU - Obál, Izabella
AU - Heintzelmann, Mia B
AU - Nix, Wilfred
AU - Camdessanché, Jean-Philippe
AU - Fuglsang-Frederiksen, Anders
AU - Tankisi, Hatice
N1 - © 2025. The Author(s).
PY - 2025/3
Y1 - 2025/3
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.
AB - 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.
KW - Aged
KW - Aged, 80 and over
KW - Amyotrophic Lateral Sclerosis/diagnosis
KW - Female
KW - Humans
KW - Male
KW - Middle Aged
KW - Motor Neurons/pathology
KW - Observer Variation
KW - Reproducibility of Results
KW - Severity of Illness Index
KW - Motor neuron disease
KW - Upper motor neuron
KW - Diagnosis
KW - Reliability
UR - http://www.scopus.com/inward/record.url?scp=85218841777&partnerID=8YFLogxK
U2 - 10.1007/s00415-025-12895-7
DO - 10.1007/s00415-025-12895-7
M3 - Journal article
C2 - 39954119
SN - 0939-1517
VL - 272
SP - 208
JO - Journal of Neurology
JF - Journal of Neurology
IS - 3
M1 - 208
ER -