Association Between Body Mass Index and Disease Severity in Chinese Spinocerebellar Ataxia Type 3 Patients

The Cerebellum
Jin-Shan YangShi-Rui Gan

Abstract

Spinocerebellar ataxia type 3 (SCA3), the most common subtype of SCA worldwide, is caused by mutation of CAG repeats expansion in ATXN3. Body mass index (BMI) is an important modulatory factor in the progression of neurodegenerative disorders such as Huntington disease and amyotrophic lateral sclerosis. However, its relevance in SCA3 is not well understood. In this study, BMI was investigated in 134 molecularly confirmed SCA3 patients and 136 healthy controls from China. The multivariable linear regression models were performed to establish the putative risk factors for BMI, and whether BMI could affect the severity of ataxia. We found that BMI was significantly lower in the case group than that in the control group. The age at onset (positive correlation) and severity of ataxia (negative correlation) were the risk factors affecting BMI. Conversely, BMI along with the disease duration, the age at onset, and the numbers of CAG repeats could also have influence on the severity of ataxia. In conclusion, SCA3 patients had lower BMI than matched controls and BMI is a predictor of disease progression in SCA3. Nutritional intervention to promote weight gain could be a promising strategy to impede SCA3 progression.

References

Aug 1, 1991·Archives of Neurology·R H MyersE D Bird
Sep 25, 1999·Neurology·J C DesportP Couratier
Nov 18, 2011·The Cerebellum·Jonas Alex Morales SauteLaura Bannach Jardim
Apr 24, 2015·Journal of Neurology·S D SüssmuthMichael Orth
Aug 11, 2017·Movement Disorders Clinical Practice·Alhassane DialloSophie Tezenas du Montcel

❮ Previous
Next ❯

Citations

Apr 11, 2020·Journal of Translational Medicine·Magda M SantanaLuís Pereira de Almeida
Feb 7, 2021·Movement Disorders : Official Journal of the Movement Disorder Society·Luis E Almaguer-MederosGeorg Auburger
Oct 30, 2021·Movement Disorders : Official Journal of the Movement Disorder Society·Holger HengelLudger Schöls

❮ Previous
Next ❯

Methods Mentioned

BETA
transgenic

Software Mentioned

R Foundation for Statistical Computing
R Project
SPSS

Related Concepts

Related Feeds

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.

ALS: Genetics

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. Here is the latest research investigating genetic alterations in this genetically heterogeneous disorder.

Ataxias (MDS)

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on ataxia here.

Ataxias

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on different types of ataxias here.

ALS: Genetics

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. ALS is a genetically heterogeneous disorder with several causative genes. Here are the latest discoveries pertaining to the genetics of this disease.

Ataxia

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on ataxia here.