Objective: This study aimed to investigate the eating habits, nutritional and metabolic status in patients with hereditary ataxia (HA) in Northeast Brazil.

Background: Studies on the nutritional profile of patients with AH report weight loss related to dysphagia, increased cellular catabolism and nutritional status related to CAG repetition time.

Method: We performed a observational case-control study. The population consisted of 38 patients with HA and 38 health volunteers, recruited from 2 neurology referral centers in Northeast of Brazil. In 2019, subjects were submitted to neurological and nutritional assessments. The Scale for the Assessment and Rating of Ataxia (SARA) was used to measure the severity of cerebellar ataxia. The anthropometric measurements were carried by measuring height and bioimpedance. We also collected data such as body weight, Basal Metabolic Rate (BMR), visceral fat level, Body Mass Index (BMI), skeletal muscle percentage and body fat percentage. We also performed a detailed nutritional physical examination, global classification assessment (SGA) and two 24-hour reminders.

Results: SARA scored 16.4 ± 7.9 for patients with dominant inheritance and 18.6 ± 8.2 for the group with recessive inheritance. Both groups had high prevalence of overweight with high BMI, high percentage of body fat and normal percentage of visceral fat. No significant correlation was found between the SARA scores ​​and the BMI or BMR variables. The average percentage of skeletal muscles in both groups was within the ideal. The BMR was similar between groups. We detected 89.5% of patients with HA were well-nourished and 10.5% malnourished, all controls were well nourihed. The daily intake values ​​of proteins, carbohydrates, cholesterol and sodium are well above the values ​​of the Dietary Reference Intakes (DRI).The nutritional physical examination of patients with HA revealed nails with hemorrhagic streaks, pale skin and presence of seborrheic dermatitis and dry mouth with little salivation.

Conclusion: HA patients are commonly well-nourished but many of them have inadequate eating habits and high percentage of body fat. We did not detect a correlation between BMI or other nutritional variable and SARA.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/eating-habits-and-nutritional-status-of-patients-with-hereditary-ataxias-in-a-northeast-brazilian-population/