Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score

Anna B Jacobsen; Gaia Fanella; Mamede de Carvalho; Martin Koltzenburg; Miguel Oliveira Santos; Bülent Cengiz; Jakob Blicher; Izabella Obál; Mia B Heintzelmann; Wilfred Nix; Jean-Philippe Camdessanché; Anders Fuglsang-Frederiksen; Hatice Tankisi

doi:10.1007/s00415-025-12895-7

Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score

Anna B Jacobsen, Gaia Fanella, Mamede de Carvalho, Martin Koltzenburg, Miguel Oliveira Santos, Bülent Cengiz, Jakob Blicher, Izabella Obál, Mia B Heintzelmann, Wilfred Nix, Jean-Philippe Camdessanché, Anders Fuglsang-Frederiksen, Hatice Tankisi

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review

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.

Original language	English
Article number	208
Book series	Journal of Neurology
Volume	272
Issue	3
Pages (from-to)	208
ISSN	0939-1517
DOIs	https://doi.org/10.1007/s00415-025-12895-7
Publication status	Published - Mar 2025

Keywords

Aged
Aged, 80 and over
Amyotrophic Lateral Sclerosis/diagnosis
Female
Humans
Male
Middle Aged
Motor Neurons/pathology
Observer Variation
Reproducibility of Results
Severity of Illness Index
Motor neuron disease
Upper motor neuron
Diagnosis
Reliability

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Jacobsen, A. B., Fanella, G., de Carvalho, M., Koltzenburg, M., Oliveira Santos, M., Cengiz, B., Blicher, J., Obál, I., Heintzelmann, M. B., Nix, W., Camdessanché, J.-P., Fuglsang-Frederiksen, A., & Tankisi, H. (2025). Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score. Journal of Neurology, 272(3), 208. Article 208. https://doi.org/10.1007/s00415-025-12895-7

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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.",

keywords = "Aged, Aged, 80 and over, Amyotrophic Lateral Sclerosis/diagnosis, Female, Humans, Male, Middle Aged, Motor Neurons/pathology, Observer Variation, Reproducibility of Results, Severity of Illness Index, Motor neuron disease, Upper motor neuron, Diagnosis, Reliability",

author = "Jacobsen, {Anna B} and Gaia Fanella and {de Carvalho}, Mamede and Martin Koltzenburg and {Oliveira Santos}, Miguel and B{\"u}lent Cengiz and Jakob Blicher and Izabella Ob{\'a}l and Heintzelmann, {Mia B} and Wilfred Nix and Jean-Philippe Camdessanch{\'e} and Anders Fuglsang-Frederiksen and Hatice Tankisi",

note = "{\textcopyright} 2025. The Author(s).",

year = "2025",

month = mar,

doi = "10.1007/s00415-025-12895-7",

language = "English",

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journal = "Journal of Neurology",

issn = "0939-1517",

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Jacobsen, AB , Fanella, G, de Carvalho, M, Koltzenburg, M, Oliveira Santos, M, Cengiz, B, Blicher, J, Obál, I, Heintzelmann, MB, Nix, W, Camdessanché, J-P, Fuglsang-Frederiksen, A & Tankisi, H 2025, 'Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score', Journal of Neurology, vol. 272, no. 3, 208, pp. 208. https://doi.org/10.1007/s00415-025-12895-7

Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score. / Jacobsen, Anna B ; Fanella, Gaia; de Carvalho, Mamede et al.
In: Journal of Neurology, Vol. 272, No. 3, 208, 03.2025, p. 208.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review

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

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 -

Variability of the Penn upper motor neuron score in amyotrophic lateral sclerosis: need for a revised score

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Keywords

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