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Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression.

ABSTRACT: Background:Spinocerebellar ataxias (SCAs) 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 SCAs (SCA1, SCA2 SCA3, and SCA6). 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 SCA1, SCA2, SCA3, or SCA6 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/m2 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.

SUBMITTER: Diallo A

PROVIDER: S-EPMC6174467 | biostudies-literature | 2017 Sep-Oct

REPOSITORIES: biostudies-literature

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Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression.

Diallo Alhassane A Jacobi Heike H Schmitz-Hübsch Tanja T Cook Arron A Labrum Robyn R Durr Alexandra A Brice Alexis A Charles Perrine P Marelli Cecilia C Mariotti Caterina C Nanetti Lorenzo L Panzeri Marta M Rakowicz Maria M Sobanska Anna A Sulek Anna A Schöls Ludger L Hengel Holger H Melegh Bela B Filla Alessandro A Antenora Antonella A Infante Jon J Berciano José J van de Warrenburg Bart P BP Timmann Dagmar D Boesch Sylvia S Pandolfo Massimo M Schulz Jörg B JB Bauer Peter P Giunti Paola P Baliko Laszlo L Parkinson Michael H MH Kang Jun-Suk JS Klockgether Thomas T Tezenas du Montcel Sophie S

Movement disorders clinical practice 20170811 5

<h4>Background</h4>Spinocerebellar ataxias (SCAs) are dominantly inherited, progressive ataxia disorders. Disease progression could be preceded by weight loss.<h4>Objectives</h4>We aimed to study the course of weight loss in patients who had the most common SCAs (SCA1, SCA2 SCA3, and SCA6). 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.<h4>Methods</h4 ...[more]

PMID: 30363449

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Project description:ObjectiveThis study aims to explore whether body mass index (BMI) level affects the executive function and hippocampal subregion volume of subjective cognitive decline (SCD).Materials and methodsA total of 111 participants were included in the analysis, including SCD (38 of normal BMI, 27 of overweight and obesity) and normal cognitive control (NC) (29 of normal BMI, 17 of overweight and obesity). All subjects underwent the Chinese version of the Stroop Color-Word Test (SCWT) to measure the executive function and a high-resolution 3D T1 structural image acquisition. Two-way ANOVA was used to examine the differences in executive function and gray matter volume in hippocampal subregions under different BMI levels between the SCD and NC.ResultThe subdimensions of executive function in which different BMI levels interact with SCD and NC include inhibition control function [SCWT C-B reaction time(s): F (1,104) = 5.732, p = 0.018], and the hippocampal subregion volume of CA1 [F (1,99) = 8.607, p = 0.004], hippocampal tail [F (1,99) = 4.077, p = 0.046], and molecular layer [F (1,99) = 6.309, p = 0.014]. After correction by Bonferroni method, the population × BMI interaction only had a significant effect on the CA1 (p = 0.004). Further analysis found that the SCWT C-B reaction time of SCD was significantly longer than NC no matter whether it is at the normal BMI level [F (1,104) = 4.325, p = 0.040] or the high BMI level [F (1,104) = 21.530, p < 0.001], and the inhibitory control function of SCD was worse than that of NC. In the normal BMI group, gray matter volume in the hippocampal subregion (CA1) of SCD was significantly smaller than that of NC [F (1,99) = 4.938, p = 0.029]. For patients with SCD, the high BMI group had worse inhibitory control function [F (1,104) = 13.499, p < 0.001] and greater CA1 volume compared with the normal BMI group [F (1,99) = 7.619, p = 0.007].ConclusionThe BMI level is related to the inhibition control function and the gray matter volume of CA1 subregion in SCD. Overweight seems to increase the gray matter volume of CA1 in the elderly with SCD, but it is not enough to compensate for the damage to executive function caused by the disease. These data provide new insights into the relationship between BMI level and executive function of SCD from the perspective of imaging.

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Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression.

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Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression.

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