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000285044 1001_ $$00000-0003-1165-9153$$aLischewski, Stella Andrea$$b0
000285044 245__ $$aAnalysis of a Modified Version of the Inventory of Non-Ataxia Signs Over 12 Years in Patients with Friedreich's Ataxia in the EFACTS Study.
000285044 260__ $$aNew York, NY$$bWiley$$c2026
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000285044 520__ $$aFriedreich's ataxia is a rare, neurodegenerative, multisystem disorder. While ataxia is a hallmark, non-ataxia signs, including muscle weakness, spasticity, and dysphagia are equally disabling. The Inventory of Non-Ataxia Signs (INAS) is a symptom list transformable to a 16-item count.To evaluate the responsiveness of a modified INAS in this population.Participants were drawn from the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS). The modified INAS count (presence/absence, 0-16 scale) and modified INAS sum (severity-weighted, 0-84 scale) were evaluated using linear mixed-models and standardized response means (SRMs). Items rare (<5%) and uncharacteristic in Friedreich's ataxia were excluded (chorea, myoclonus, fasciculations, resting tremor, rigidity) RESULTS: A total of 1129 participants (mean age, 32.3 years) were assessed for up to 12 years. The mean modified INAS count was 4.6 (±2.2) and modified INAS sum 15.1 (± 9.9). Both correlated strongly with existing outcome measures. Longitudinally, the modified INAS count increased by 0.13 points/year (95% CI 0.12, 0.14; P < 0.001) and modified INAS sum by 0.68 points/year (95% CI 0.64, 0.72; P < 0.001). The modified INAS sum demonstrated greater responsiveness, with SRMs of 0.26, 0.38, 0.53, and 0.80 at 1, 2, 3, and 5 years, respectively, compared with 0.16, 0.27, 0.31, and 0.46 for the modified INAS count. In non-ambulatory patients and children, responsiveness of the modified INAS sum was higher (SRM 0.82 and 1.7 at 5 years, respectively).The modified INAS sum showed good responsiveness over 5 years but not over 1-3 years. It may supplement existing outcome measures, contributing to holistic assessment of this multisystem disease, especially in non-ambulatory patients, in whom ataxia-focused measures may show ceiling effects, and children, who typically progress faster. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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000285044 650_7 $$2Other$$aFriedreich's ataxia
000285044 650_7 $$2Other$$alongitudinal
000285044 650_7 $$2Other$$anon‐ataxia symptoms
000285044 650_2 $$2MeSH$$aHumans
000285044 650_2 $$2MeSH$$aFriedreich Ataxia: diagnosis
000285044 650_2 $$2MeSH$$aFriedreich Ataxia: physiopathology
000285044 650_2 $$2MeSH$$aFriedreich Ataxia: complications
000285044 650_2 $$2MeSH$$aMale
000285044 650_2 $$2MeSH$$aFemale
000285044 650_2 $$2MeSH$$aAdult
000285044 650_2 $$2MeSH$$aMiddle Aged
000285044 650_2 $$2MeSH$$aYoung Adult
000285044 650_2 $$2MeSH$$aSeverity of Illness Index
000285044 650_2 $$2MeSH$$aAdolescent
000285044 650_2 $$2MeSH$$aChild
000285044 7001_ $$aDogan, Imis$$b1
000285044 7001_ $$00000-0003-3508-4788$$aGiunti, Paola$$b2
000285044 7001_ $$aParkinson, Michael H$$b3
000285044 7001_ $$00000-0003-2405-3564$$aMariotti, Caterina$$b4
000285044 7001_ $$aDurr, Alexandra$$b5
000285044 7001_ $$00000-0002-4864-6287$$aEwenczyk, Claire$$b6
000285044 7001_ $$aBoesch, Sylvia$$b7
000285044 7001_ $$aNachbauer, Wolfgang$$b8
000285044 7001_ $$0P:(DE-2719)2810704$$aKlopstock, Thomas$$b9$$udzne
000285044 7001_ $$0P:(DE-2719)2812141$$aStendel, Claudia$$b10
000285044 7001_ $$ade Rivera Garrido, Francisco Javier Rodríguez$$b11
000285044 7001_ $$0P:(DE-2719)2810795$$aSchöls, Ludger$$b12$$udzne
000285044 7001_ $$0P:(DE-2719)9000074$$aFleszar, Zofia$$b13$$udzne
000285044 7001_ $$0P:(DE-2719)2810314$$aKlockgether, Thomas$$b14
000285044 7001_ $$0P:(DE-2719)9001510$$aGrobe-Einsler, Marcus$$b15
000285044 7001_ $$0P:(DE-2719)2811662$$aGiordano, Ilaria$$b16$$udzne
000285044 7001_ $$aRai, Myriam$$b17
000285044 7001_ $$aPandolfo, Massimo$$b18
000285044 7001_ $$0P:(DE-2719)2811564$$aJacobi, Heike$$b19
000285044 7001_ $$aHilgers, Ralf-Dieter$$b20
000285044 7001_ $$aSchulz, Jörg B$$b21
000285044 7001_ $$00000-0002-9730-9228$$aReetz, Kathrin$$b22
000285044 7001_ $$aGroup, EFACTS Study$$b23$$eCollaboration Author
000285044 7001_ $$aIndelicato, Elisabetta$$b24$$eContributor
000285044 7001_ $$aAmprosi, Matthias$$b25$$eContributor
000285044 7001_ $$aGellera, Cinzia$$b26$$eContributor
000285044 7001_ $$aMongelli, Alessia$$b27$$eContributor
000285044 7001_ $$aCastaldo, Anna$$b28$$eContributor
000285044 7001_ $$aFichera, Mario$$b29$$eContributor
000285044 7001_ $$aBertini, Enrico$$b30$$eContributor
000285044 7001_ $$aVasco, Gessica$$b31$$eContributor
000285044 7001_ $$aBiet, Marie$$b32$$eContributor
000285044 7001_ $$aMonin, Marie Lorraine$$b33$$eContributor
000285044 7001_ $$aHoltbernd, Florian$$b34$$eContributor
000285044 7001_ $$aBrcina, Nikolina$$b35$$eContributor
000285044 7001_ $$aHohenfeld, Christian$$b36$$eContributor
000285044 7001_ $$aRadelfahr, Florentine$$b37$$eContributor
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000285044 7001_ $$aKoutsis, Georgios$$b40$$eContributor
000285044 7001_ $$aBreza, Marianthi$$b41$$eContributor
000285044 7001_ $$aPalau, Francesc$$b42$$eContributor
000285044 7001_ $$aO'Callaghan, Mar$$b43$$eContributor
000285044 7001_ $$aThomas-Black, Gilbert$$b44$$eContributor
000285044 7001_ $$aManso, Katarina$$b45$$eContributor
000285044 7001_ $$aSolanky, Nita$$b46$$eContributor
000285044 7001_ $$aLabrum, Robyn$$b47$$eContributor
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