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Record W4413339166 · doi:10.1002/mds.70006

Spastic Ataxia Composite ( <scp>SPAXCOM</scp> ): A Scale to Evaluate the Progression of Subjects with Spasticity and Ataxia

2025· article· en· W4413339166 on OpenAlexaffabout
Cécile Di Folco, Charlotte Dubec‐Fleury, Andreas Traschütz, Christoph Keßler, Selina Reich, Cynthia Gagnon, Isabelle Lessard, Xavier Rodrigue, Sirio Cocozza, Sara Satolli, Filippo M. Santorelli, Alexandra Dürr, Anna Heinzmann, Bart P. van de Warrenburg, Ilse H. J. Willemse, A. Nazlı Başak, Atay Vural, Bernard Brais, Stephan Klebe, Rita Horváth, Rebecca Schüle, Matthis Synofzik, Sophie Tézenas du Montcel

Bibliographic record

VenueMovement Disorders · 2025
Typearticle
Languageen
FieldNeuroscience
TopicHereditary Neurological Disorders
Canadian institutionsCentres Intégré Universitaires de Santé et de Services SociauxCégep de JonquièreMontreal Neurological Institute and HospitalMcGill UniversityCentre Intégré Universitaire de Santé et de Services Sociaux du Saguenay–Lac-Saint-JeanUniversité de Sherbrooke
FundersNIHR Cambridge Biomedical Research CentreNational Medical Research CouncilMedical Research CouncilMinistero della SaluteElse Kröner-Fresenius-StiftungNational Institute for Health and Care ResearchBundesministerium für Bildung und ForschungEberhard Karls Universität TübingenHORIZON EUROPE Framework ProgrammeWellcome TrustAgence Nationale de la RechercheAtaxia UKDeutsche ForschungsgemeinschaftUK Research and InnovationDepartment of Health and Social CareZonMwLifeArc
KeywordsAtaxiaSpasticitySpasticRating scaleSpinocerebellar ataxiaMedicinePhysical therapyPhysical medicine and rehabilitationModified Ashworth scalePsychologyCerebral palsyPsychiatryDevelopmental psychology

Abstract

fetched live from OpenAlex

BACKGROUND: Current clinical scales that track disease progression are more tailored to spasticity or ataxia, with limited sensitivity to change. OBJECTIVES: The aim was to develop a sensitive and valid scale specifically geared towards optimized sensitivity to change and adapted to patients presenting with both spasticity and ataxia. METHODS: Longitudinal data from 127 spastic paraplegia type 7 (SPG7) and 112 autosomal recessive spastic ataxia Charlevoix-Saguenay (ARSACS) patients were collected within the multicenter PROSPAX study. Sensitivity to change over 2 years of 30 items from the Scale for the Rating and Assessment of Ataxias (SARA), Spastic Paraplegia Rating Scale (SPRS), and the Activities of Daily Living subscale of the Friedreich's Ataxia Rating Scale (FARS-ADL) was evaluated. Items that demonstrated the highest sensitivity to change were used to build the Spastic Ataxia Composite scale (SPAXCOM). RESULTS: With seven items, the SPAXCOM showed an effect size of 0.71, higher than reference scales (SARA: 0.43, SPRS: 0.42, FARS-ADL: 0.27). The SPAXCOM had a similar sensitivity to change for both genotypes and was more sensitive in participants with a SARA lower than 10 and within the intermediate disease stage (FARS-Disease Staging: 2-3.5). The SPAXCOM showed a high correlation with disease duration (r = 0.67 [0.60; 0.72]) and disease stage (r = 0.86 [0.83; 0.89]). Perception of improvement, stagnation, and worsening were associated with a mean yearly SPAXCOM change of 0.44 (-0.14; 1.01), 0.61 (0.19; 1.03), and 1.22 (0.96; 1.49), respectively. CONCLUSION: The SPAXCOM is more sensitive to change and homogeneous across genotypes than the reference scales, allowing a reduction of the required sample size in future clinical trials. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.274
Threshold uncertainty score0.822

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.001
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0010.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.015
GPT teacher head0.267
Teacher spread0.253 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations1
Published2025
Admission routes2
Has abstractyes

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