Phase angle (PhA) and bioelectrical impedance vector analysis (BIVA) have emerged as valuable tools for assessing nutritional status and prognosis in various patient populations, but there is a lack of studies in rare neurodegenerative diseases. The purpose of this cross-sectional study was to investigate these bioelectrical parameters in patients with amyotrophic lateral sclerosis (ALS), compared with healthy peers. The tetrapolar impedance method was applied and bioimpedance analysis (BIA) was performed. Bioelectrical parameters were obtained (Resistance—R; Reactance—Xc) or calculated (PhA; BIVA). For BIVA, bivariate vectors and confidence ellipses were graphically represented. In addition, R and Xc were used to determine body composition (BC) (Fat Mass—FM; Fat-Free Mass—FFM; and Total Body Water—TBW). In this study, 40 participants were divided into two groups: case group (n = 20, ALS patients) and control group (n = 20, healthy subjects. Our main results showed that ALS patients presented low levels of BMI, FFM, R, and Xc adjusted by height (R/H, Xc/H), hydration, and cellularity, compared to the healthy subjects. Our findings highlight BC and bioelectrical parameters, including PhA and BIVA, as valuable indicators of nutrition status, which should be implemented in the nutrition care process of ALS patients during the disease course.

Anthropometry and body composition evaluation in boys with Duchenne muscular dystrophy (DMD) are challenging, but crucial methods to evaluate the nutritional status, and better anthropometric reference values and body composition predictive equations are needed for this population. Based on these aspects, this study aimed to investigate the hypothesis that changes in anthropometric parameters and body composition of boys with DMD occur according to age. A cross-sectional study with 49 individuals diagnosed with DMD at the neurological outpatient facility at the Onofre Lopes University Hospital in Natal, Brazil, was performed between September 2016 and March 2019. These individuals underwent anthropometric and body composition evaluation. According to age, the participants were divided into four groups: G1 (2.6 - 8.2y), G2 (8.5 - 10.8y), G3 (11.0 - 14.0y), and G4 (15.9 - 23.0y). The parameters weight-for-age (W/A) (p=0.025), tricipital skinfold (TSF) (p=0.027), adductor pollicis muscle (p=0.041), and corrected arm muscle area (cAMA) (p=0.005) were different among the groups. Regarding anthropometric parameters, was prevalence in the categories of appropriate W/A and a height-for-age (H/A), and eutrophy for body mass index-for-age (BMI/A). For the TSF, there was a higher frequency of severe malnutrition or obesity. The cAMA indicated severe malnutrition in most individuals. As for %FM, high adiposity was more frequent, increasing over age groups (G1 to G4). The boys with DMD presented different patterns of anthropometric and body composition parameters. An increase of fat mass and a decrease of lean mass with age/disease progression were observed.

The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) is a standard tool for evaluating functional decline in patients with ALS. Despite its clinical value, administration by healthcare professionals can be time-consuming and resource intensive. To assess the reliability and feasibility of a self-administration version of the ALSFRS-R as an alternative for use in clinical and research settings. The present was an observational, analytical, prospective, single-center study involving ALS patients followed at the Neurology Outpatient Clinic of the Hospital Universitário Onofre Lopes (HUOL). Three independent assessments of the ALSFRS-R were conducted: one self-administered version and two interviewer-administered versions performed by different researchers during face-to-face consultations. Interrater reliability was assessed using intraclass correlation coefficients (ICCs), and agreement among the three versions was analyzed using Bland-Altman plots. A total of 43 participants were included in the study, with a mean age of 57 ± 11.67 years. The ICCs indicated high reliability for both the total ALSFRS-R score and its functional domains. Linear regression analyses demonstrated strong agreement between the two researchers (R2 = 0.98, p < 0.001), as well as between each researcher and the self-administered version (R2 = 0.90 and 0.88, respectively). Bland-Altman analyses showed minimal bias and acceptable limits of agreement across all comparisons. The self-administered version of the ALSFRS-R demonstrated high reliability and strong agreement with the researcher-administered versions, supporting its potential use for remote monitoring of ALS patients. Nonetheless, it should not replace professional assessments in clinical trial settings. Further research is warranted to validate its applicability in broader clinical contexts and diverse patient populations.

Individuals with Duchenne muscular dystrophy (DMD) are prone to nutritional imbalances, and zinc deficiency may contribute to impaired bone health. This study evaluated serum zinc status and the effects of oral supplementation on bone parameters in DMD. In this quasi-experimental before-and-after study, 34 patients were assessed at three time points over eight months. Eligible participants who met the inclusion criteria and agreed to participate received the proposed interventions during routine follow-up at the Neurology outpatient clinic. Anthropometry, dietary intake, bone mineral density (BMD), bone mineral content (BMC), and serum zinc were measured; supplementation (5–15 mg/day) was provided for four months. Baseline zinc deficiency was observed in 36.7% of participants. No significant overall changes were detected. Stratified analyses revealed a modest increase in total body BMD among individuals with adequate baseline BMD (p = 0.02). As this finding emerged from a subgroup analysis, it should be interpreted cautiously, and the potential contribution of physiological growth to the observed change cannot be excluded. In addition, zinc-deficient participants showed a significant rise in serum zinc levels (p = 0.008). These findings suggest that the response to zinc supplementation may vary according to baseline nutritional and skeletal status and underscore the relevance of micronutrient monitoring in individuals with DMD. Trial registration: The trial was also registered in the Brazilian Registry of Clinical Trials under the code RBR-7cfdxm, approved on 14 June 2018.

Background. The study hypothesized that zinc supplementation would increase or preserve lean body mass in Duchenne muscular dystrophy (DMD) patients. Therefore, we aimed to evaluate the body composition of DMD patients before and after zinc supplementation. Materials and Methods. The study is a clinical trial comprising 21 boys diagnosed with DMD. Dietary intake parameters were evaluated before zinc supplementation. Serum zinc levels, anthropometry, and body composition were measured in three moments, four months apart. The patients received 5, 10, or 15 mg of zinc bis‐glycine supplementation according to age as an oral solution daily for four months. The sample was distributed into two groups according to serum zinc status: zinc deficiency (G1) or adequate zinc (G2). Results. There was a significant difference in lean body mass between the groups: zinc deficiency (G1) or adequate zinc (G2), at three times (p = 0.041, 0.016, and 0.009, respectively). After oral zinc supplementation, serum zinc levels were not different between groups. We did not observe differences when associating the parameters between times and groups. Conclusion. Zinc supplementation was able to maintain lean body mass and fat mass in patients with DMD with previous deficiencies. Therefore, it is necessary to have a prior screening of serum zinc levels to observe changes after supplementation.