Nutritional status and eating habits of patients with hereditary ataxias: a case–control study

ABSTRACT

Introduction

Hereditary Ataxias (HAs) comprise a wide spectrum of genetically determined neurodegenerative diseases with progressive ataxia as the main symptom. Few studies have evaluated nutritional profile in HA patients and most of these focused on specific ataxia subtypes. The objectives of this study were: (1) to investigate whether hereditary ataxias were associated with changes in energy expenditure, body composition and dietary intake; (2) to verify differences in these variables according to ataxia subtype, sex, age, and disease severity.

Methods

Thirty-eight hereditary ataxia patients from two neurology centers in Northeastern Brazil and 38 controls were evaluated. Body composition was assessed with bio-impedance analysis and dietary intake was estimated with a validated questionnaire (24-hour dietary recall).

Results

Mean body mass index (BMI) was lower in HA compared to controls (p = 0.032). Hereditary ataxia patients showed lower protein intake, higher frequency of dysphagia and higher incidence of nausea and diarrhea. The difference in average estimated caloric intake did not reach statistical significance (2359kcal ± 622 in patients × 2713kcal ± 804 in controls, p = 0.08). Disease severity measured by the SARA scale was not associated with BMI, nor was ataxia subtype (autosomal dominant × non-autosomal dominant ataxias).

Conclusion

Hereditary ataxia patients have lower BMI compared to healthy controls. There was no difference in this cohort between dominant or non-dominant ataxia regarding BMI. Weight loss may be a common finding among hereditary ataxias and may affect the quality of life in these patients.

KEYWORDS:

• Hereditary ataxia
• feeding behavior
• nutritional assessment
• body composition
• diet

Disclosure statement

No potential conflict of interest was reported by the author(s).

Ethical statement

Patients signed an informed consent form and allowed publication of this data.

Data availability statement

All materials and data used in this study will be made available upon request.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.

Notes on contributors

Camila Gonçalves Monteiro Carvalho

Camila Gonçalves Monteiro Carvalho - Nutritionist.

Paulo Ribeiro Nóbrega

Paulo Ribeiro Nóbrega - Neurologist, researcher in the filed of neurogenetics.

Stephanie Suzanne de Oliveira Scott

Stephanie Suzanne de Oliveira Scott - Neurologist, researcher in the fied of neurogenetics.

Deborah Moreira Rangel

Deborah Moreira Rangel - Neurologist, researcher in the field of neurogenetics.

Danyela Martins Bezerra Soares

Danyela Martins Bezerra Soares - Medical student.

Carla Soraya Costa Maia

Carla Soraya Costa Maia - Nutritionist, associate professor at Universidade Estadual do Ceará.

Pedro Braga-Neto

Pedro Braga-Neto - associate professor of neurology at Universidade Federal do Ceará and Universidade Estadual do Ceará.