In:
Movement Disorders Clinical Practice, Wiley, Vol. 4, No. 5 ( 2017-09), p. 689-697
Abstract:
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.
Type of Medium:
Online Resource
ISSN:
2330-1619
,
2330-1619
DOI:
10.1002/mdc3.2017.4.issue-5
Language:
English
Publisher:
Wiley
Publication Date:
2017
detail.hit.zdb_id:
2772809-2
