Evidence map›Paper›PMID 39954119›Full record

ArticleJournal of neurology2025

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 and 3 more

Abstract read
In one paragraph

Article in Journal of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

13 authors.

Anna B JacobsenDepartment of Clinical Neurophysiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 165, J209, 8200, Aarhus, Denmark. annajase@rm.dk.ORCID http://orcid.org/0009-0002-8110-3636
Gaia FanellaDepartment of Clinical Neurophysiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 165, J209, 8200, Aarhus, Denmark.
Mamede de CarvalhoInstitute of Physiology, Centro de Estudos Egas Moniz, Faculdade de Medicina, Instituto de Medicina Molecular João Lobo Antunes, Universidade de Lisboa, Lisbon, Portugal.
Martin KoltzenburgDepartment of Clinical Neurophysiology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Miguel Oliveira SantosInstitute of Physiology, Centro de Estudos Egas Moniz, Faculdade de Medicina, Instituto de Medicina Molecular João Lobo Antunes, Universidade de Lisboa, Lisbon, Portugal.
Bülent CengizDepartment of Neurology, Faculty of Medicine, Gazi University, Beşevler, Ankara, Turkey.
Jakob BlicherDepartment of Neurology, Aalborg University Hospital, Aalborg, Denmark.
Izabella ObálDepartment of Neurology, Aalborg University Hospital, Aalborg, Denmark.
Mia B HeintzelmannDepartment of Clinical Medicine - Center of Functionally Integrative Neuroscience, Aarhus, Denmark.
Wilfred NixDepartment of Neurology, Johannes Gutenberg University, Mainz, Germany.
Jean-Philippe CamdessanchéDepartment of Neurology, University Hospital of Saint-Etienne, Saint-Etienne, France.
Anders Fuglsang-FrederiksenDepartment of Clinical Neurophysiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 165, J209, 8200, Aarhus, Denmark.
Hatice TankisiDepartment of Clinical Neurophysiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 165, J209, 8200, Aarhus, Denmark.

Funding

Aage og Johanne Louis-Hansens Fond J.nr.23-2B-12533Lundbeck Foundation R346-2020-1946Lundbeck Foundation R392-2022-699
6 · The paper itself

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.

Indexed as

Amyotrophic Lateral SclerosisMotor NeuronsSeverity of Illness IndexAgedAged, 80 and overFemaleHumansMaleMiddle AgedObserver VariationReproducibility of ResultsDiagnosisMotor neuron diseaseReliabilityUpper motor neuron

Identifiers

PMID39954119
PMCPMC11829849

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.