| Title: |
Spastic Ataxia Composite ( SPAXCOM ): A Scale to Evaluate the Progression of Subjects with Spasticity and Ataxia |
| Authors: |
Di Folco, Cécile; Dubec‐fleury, Charlotte; Traschütz, Andreas; Kessler, Christoph; Reich, Selina; Gagnon, Cynthia; Lessard, Isabelle; Rodrigue, Xavier; Cocozza, Sirio; Satolli, Sara; Santorelli, Filippo, M; Durr, Alexandra; Heinzmann, Anna; van de Warrenburg, Bart, P; Willemse, Ilse H.J.; Başak, A. Nazli; Vural, Atay; Brais, Bernard; Klebe, Stephan; Horvath, Rita; Schüle, Rebecca; Synofzik, Matthis; Tezenas Du Montcel, Sophie |
| Contributors: |
Algorithms, models and methods for images and signals of the human brain = Algorithmes, modèles et méthodes pour les images et les signaux du cerveau humain ICM Paris (ARAMIS); Institut du Cerveau = Paris Brain Institute (ICM); Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Centre Inria de Sorbonne Université; Centre Inria de Paris; Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre Inria de Paris; Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria); ICM; Uniwersytet Warszawski Polska = University of Warsaw Poland = Université de Varsovie Pologne (UW); University Children's Hospital of Tübingen; Partenaires INRAE; Universitätsklinikum Tübingen - University Hospital of Tübingen; Eberhard Karls Universität Tübingen = University of Tübingen; Department of Computer and Information Science - Linköping University; Linköping University (LIU); Deutsches Zentrum für Neurodegenerative Erkrankungen Ulm (DZNE); German Research Center for Neurodegenerative Diseases - Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE); Faculté de médecine et des sciences de la santé Sherbrooke (UdeS); Université de Sherbrooke = University of Sherbrooke Sherbrooke (UdeS); Cégep de Jonquière; Centre Intégré Universitaire de Santé et de Services Sociaux de la Capitale Nationale; Icahn School of Medicine at Mount Sinai New York (MSSM); University of Naples Federico II = Università degli studi di Napoli Federico II (UNINA); Dipartimento di Ingegneria e Geologia Chieti; Universita' degli Studi "G. d'Annunzio" Chieti-Pescara (UNICH); IRCCS Fondazione Stella Maris Pisa; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS); Radboud University Medical Center Nijmegen (RadboudUMC); Koç University; Montreal Neurological Institute and Hospital; McGill University = Université McGill Montréal, Canada; University Hospital Freiburg; Department of Clinical Neurosciences Cambridge; University of Cambridge Cambridge, UK (CAM); Hertie Institute for Clinical Brain Research Tubingen |
| Source: |
ISSN: 0885-3185. |
| Publisher Information: |
CCSD; Wiley |
| Publication Year: |
2025 |
| Subject Terms: |
[SDV]Life Sciences [q-bio]; [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] |
| Description: |
International audience ; Abstract 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. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1002/mds.70006 |
| Availability: |
https://inria.hal.science/hal-05229818; https://inria.hal.science/hal-05229818v1/document; https://inria.hal.science/hal-05229818v1/file/Movement%20Disorders%20-%202025%20-%20Di%20Folco%20-%20Spastic%20Ataxia%20Composite%20%20SPAXCOM%20%20%20A%20Scale%20to%20Evaluate%20the%20Progression%20of%20Subjects.pdf; https://doi.org/10.1002/mds.70006 |
| Rights: |
http://creativecommons.org/licenses/by/ ; info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.8D6E2960 |
| Database: |
BASE |