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Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression
Author(s) -
Diallo Alhassane,
Jacobi Heike,
SchmitzHübsch Tanja,
Cook Arron,
Labrum Robyn,
Durr Alexandra,
Brice Alexis,
Charles Perrine,
Marelli Cecilia,
Mariotti Caterina,
Nanetti Lorenzo,
Panzeri Marta,
Rakowicz Maria,
Sobanska Anna,
Sulek Anna,
Schöls Ludger,
Hengel Holger,
Melegh Bela,
Filla Alessandro,
Antenora Antonella,
Infante Jon,
Berciano José,
Warrenburg Bart P.,
Timmann Dagmar,
Boesch Sylvia,
Pandolfo Massimo,
Schulz Jörg B.,
Bauer Peter,
Giunti Paola,
Baliko Laszlo,
Parkinson Michael H.,
Kang JunSuk,
Klockgether Thomas,
Tezenas du Montcel Sophie
Publication year - 2017
Publication title -
movement disorders clinical practice
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.754
H-Index - 18
ISSN - 2330-1619
DOI - 10.1002/mdc3.12522
Subject(s) - spinocerebellar ataxia , body mass index , medicine , cohort , weight loss , ataxia , cohort study , prospective cohort study , disease , obesity , psychiatry
Background Spinocerebellar ataxias ( SCA s) are dominantly inherited, progressive ataxia disorders. Disease progression could be preceded by weight loss. Objectives We aimed to study the course of weight loss in patients who had the most common SCA s ( SCA 1, SCA 2 SCA 3, and SCA 6). Additional objectives were to identify subgroups of weight evolution, to determine the factors influencing these evolutions, and to assess the impact of these evolutions on disease progression. Methods In total, 384 patients from the EUROSCA prospective cohort study were analyzed who had SCA 1, SCA 2, SCA 3, or SCA 6 and at least 3 measurements of weight. Age was used as a time scale. Clinical outcomes were body mass index ( BMI ) and the Scale for the Assessment and Rating Ataxia ( SARA ), with scores ranging from 0 to 40. We used a linear mixed model to analyze the course of BMI and a latent class mixed model to identify subgroup BMI evolution. Results Overall, BMI declined over time (−0.11 ± 0.03 kg/m 2 per decade; P = 0.0009). Three subgroups of BMI evolution were identified: “decreasing BMI ” (n = 88; 23%), “increasing BMI ” (n = 70; 18%) and “stable BMI ” (n = 226; 59%). Patients in the decreasing BMI group were more severely affected at baseline with higher SARA scores and a higher frequency of non‐ataxia signs (especially motor symptoms) compared with those in the other groups. Weight loss was associated with faster disease progression (5.7 ± 0.7 SARA points per decade; P = 0.036). Conclusions The current data have substantial implications for the design of future interventional studies in SCA , as they provide a basis for patient stratification and emphasize the usefulness of BMI as a biomarker for monitoring disease progression.