Body Mass Index Is Significantly Associated With Disease Severity in Spinocerebellar Ataxia Type 2 Patients
| Autor: | Georg Auburger, Luis E. Almaguer-Mederos, Maria Velázquez-Garcés, Ilbedis Pérez-Ávila, Ricardo Hechavarría-Pupo, Dennis Almaguer-Gotay, Annelié Rodríguez-Estupiñán, Raúl Aguilera-Rodríguez |
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| Rok vydání: | 2021 |
| Předmět: |
Male
0301 basic medicine medicine.medical_specialty Ataxia Context (language use) Disease Severity of Illness Index Body Mass Index 03 medical and health sciences 0302 clinical medicine Weight loss Internal medicine medicine Humans Spinocerebellar Ataxias business.industry medicine.disease Cross-Sectional Studies 030104 developmental biology Neurology Case-Control Studies Spinocerebellar ataxia Biomarker (medicine) Female Neurology (clinical) medicine.symptom Age of onset business Body mass index 030217 neurology & neurosurgery |
| Zdroj: | Movement Disorders. 36:1372-1380 |
| ISSN: | 1531-8257 0885-3185 |
| DOI: | 10.1002/mds.28498 |
| Popis: | Background Spinocerebellar ataxia type 2 is a progressive neurodegenerative disorder due to an unstable expansion of a CAG repeat in the ATXN2 gene. Although weight loss has been associated with disease progression in several neurodegenerative conditions, it has been barely assessed in patients with spinocerebellar ataxia type 2. Objective The objective of this study was to test whether body mass index is altered in patients with spinocerebellar ataxia type 2 with varying expansion sizes from early to late disease stages. Methods A cross-sectional case-control study was performed, which included 222 clinically and molecularly diagnosed patients and 214 sex- and age-matched healthy individuals. ATXN2 genotypes and sex were considered as risk factors. Clinical outcomes included the body mass index, age at onset, disease duration, Scale for the Assessment and Rating of Ataxia score, disease stage, dysphagia, and progression rate. Multiple linear regression models were generated. Results Body mass index was significantly decreased in male patients, but not in female patients, relative to control subjects. In addition to sex, body mass index was significantly associated with age at onset and progression rate. Conversely, body mass index, along with repeat length in ATXN2 expanded alleles and disease duration, was associated with Scale for the Assessment and Rating of Ataxia score. In addition, body mass index, along with the age at onset and the repeat length in ATXN2 normal and expanded alleles, has a significant influence on progression rate. Conclusions Body mass index might be a useful biomarker of disease severity, particularly in male patients with spinocerebellar ataxia type 2 in the context of nutritional interventions or clinical trials assessing the efficacy of promising new drugs. © 2021 International Parkinson and Movement Disorder Society. |
| Databáze: | OpenAIRE |
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