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| Home > In process > Analysis of a Modified Version of the Inventory of Non-Ataxia Signs Over 12 Years in Patients with Friedreich's Ataxia in the EFACTS Study. > print |
| 001 | 285044 | ||
| 005 | 20260209103229.0 | ||
| 024 | 7 | _ | |a 10.1002/mds.70084 |2 doi |
| 024 | 7 | _ | |a pmid:41070559 |2 pmid |
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| 024 | 7 | _ | |a 0885-3185 |2 ISSN |
| 024 | 7 | _ | |a 1531-8257 |2 ISSN |
| 037 | _ | _ | |a DZNE-2026-00168 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a Lischewski, Stella Andrea |0 0000-0003-1165-9153 |b 0 |
| 245 | _ | _ | |a Analysis of a Modified Version of the Inventory of Non-Ataxia Signs Over 12 Years in Patients with Friedreich's Ataxia in the EFACTS Study. |
| 260 | _ | _ | |a New York, NY |c 2026 |b Wiley |
| 336 | 7 | _ | |a article |2 DRIVER |
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| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1770629143_24270 |2 PUB:(DE-HGF) |
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| 520 | _ | _ | |a Friedreich'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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| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de |
| 650 | _ | 7 | |a Friedreich's ataxia |2 Other |
| 650 | _ | 7 | |a longitudinal |2 Other |
| 650 | _ | 7 | |a non‐ataxia symptoms |2 Other |
| 650 | _ | 2 | |a Humans |2 MeSH |
| 650 | _ | 2 | |a Friedreich Ataxia: diagnosis |2 MeSH |
| 650 | _ | 2 | |a Friedreich Ataxia: physiopathology |2 MeSH |
| 650 | _ | 2 | |a Friedreich Ataxia: complications |2 MeSH |
| 650 | _ | 2 | |a Male |2 MeSH |
| 650 | _ | 2 | |a Female |2 MeSH |
| 650 | _ | 2 | |a Adult |2 MeSH |
| 650 | _ | 2 | |a Middle Aged |2 MeSH |
| 650 | _ | 2 | |a Young Adult |2 MeSH |
| 650 | _ | 2 | |a Severity of Illness Index |2 MeSH |
| 650 | _ | 2 | |a Adolescent |2 MeSH |
| 650 | _ | 2 | |a Child |2 MeSH |
| 700 | 1 | _ | |a Dogan, Imis |b 1 |
| 700 | 1 | _ | |a Giunti, Paola |0 0000-0003-3508-4788 |b 2 |
| 700 | 1 | _ | |a Parkinson, Michael H |b 3 |
| 700 | 1 | _ | |a Mariotti, Caterina |0 0000-0003-2405-3564 |b 4 |
| 700 | 1 | _ | |a Durr, Alexandra |b 5 |
| 700 | 1 | _ | |a Ewenczyk, Claire |0 0000-0002-4864-6287 |b 6 |
| 700 | 1 | _ | |a Boesch, Sylvia |b 7 |
| 700 | 1 | _ | |a Nachbauer, Wolfgang |b 8 |
| 700 | 1 | _ | |a Klopstock, Thomas |0 P:(DE-2719)2810704 |b 9 |u dzne |
| 700 | 1 | _ | |a Stendel, Claudia |0 P:(DE-2719)2812141 |b 10 |
| 700 | 1 | _ | |a de Rivera Garrido, Francisco Javier Rodríguez |b 11 |
| 700 | 1 | _ | |a Schöls, Ludger |0 P:(DE-2719)2810795 |b 12 |u dzne |
| 700 | 1 | _ | |a Fleszar, Zofia |0 P:(DE-2719)9000074 |b 13 |u dzne |
| 700 | 1 | _ | |a Klockgether, Thomas |0 P:(DE-2719)2810314 |b 14 |
| 700 | 1 | _ | |a Grobe-Einsler, Marcus |0 P:(DE-2719)9001510 |b 15 |
| 700 | 1 | _ | |a Giordano, Ilaria |0 P:(DE-2719)2811662 |b 16 |u dzne |
| 700 | 1 | _ | |a Rai, Myriam |b 17 |
| 700 | 1 | _ | |a Pandolfo, Massimo |b 18 |
| 700 | 1 | _ | |a Jacobi, Heike |0 P:(DE-2719)2811564 |b 19 |
| 700 | 1 | _ | |a Hilgers, Ralf-Dieter |b 20 |
| 700 | 1 | _ | |a Schulz, Jörg B |b 21 |
| 700 | 1 | _ | |a Reetz, Kathrin |0 0000-0002-9730-9228 |b 22 |
| 700 | 1 | _ | |a Group, EFACTS Study |b 23 |e Collaboration Author |
| 700 | 1 | _ | |a Indelicato, Elisabetta |b 24 |e Contributor |
| 700 | 1 | _ | |a Amprosi, Matthias |b 25 |e Contributor |
| 700 | 1 | _ | |a Gellera, Cinzia |b 26 |e Contributor |
| 700 | 1 | _ | |a Mongelli, Alessia |b 27 |e Contributor |
| 700 | 1 | _ | |a Castaldo, Anna |b 28 |e Contributor |
| 700 | 1 | _ | |a Fichera, Mario |b 29 |e Contributor |
| 700 | 1 | _ | |a Bertini, Enrico |b 30 |e Contributor |
| 700 | 1 | _ | |a Vasco, Gessica |b 31 |e Contributor |
| 700 | 1 | _ | |a Biet, Marie |b 32 |e Contributor |
| 700 | 1 | _ | |a Monin, Marie Lorraine |b 33 |e Contributor |
| 700 | 1 | _ | |a Holtbernd, Florian |b 34 |e Contributor |
| 700 | 1 | _ | |a Brcina, Nikolina |b 35 |e Contributor |
| 700 | 1 | _ | |a Hohenfeld, Christian |b 36 |e Contributor |
| 700 | 1 | _ | |a Radelfahr, Florentine |b 37 |e Contributor |
| 700 | 1 | _ | |a Bischoff, Almut |0 P:(DE-2719)9003293 |b 38 |e Contributor |u dzne |
| 700 | 1 | _ | |a Hayer, Stefanie |0 P:(DE-2719)2813263 |b 39 |e Contributor |u dzne |
| 700 | 1 | _ | |a Koutsis, Georgios |b 40 |e Contributor |
| 700 | 1 | _ | |a Breza, Marianthi |b 41 |e Contributor |
| 700 | 1 | _ | |a Palau, Francesc |b 42 |e Contributor |
| 700 | 1 | _ | |a O'Callaghan, Mar |b 43 |e Contributor |
| 700 | 1 | _ | |a Thomas-Black, Gilbert |b 44 |e Contributor |
| 700 | 1 | _ | |a Manso, Katarina |b 45 |e Contributor |
| 700 | 1 | _ | |a Solanky, Nita |b 46 |e Contributor |
| 700 | 1 | _ | |a Labrum, Robyn |b 47 |e Contributor |
| 773 | _ | _ | |a 10.1002/mds.70084 |g Vol. 41, no. 1, p. 200 - 211 |0 PERI:(DE-600)2041249-6 |n 1 |p 200 - 211 |t Movement disorders |v 41 |y 2026 |x 0885-3185 |
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