Impact of weight loss and disease progression on survival in ALS: Insights from a multidisciplinary care center Mário Emílio Teixeira Dourado Junior, Laura Carvalheira Dourado, Glauciane Costa Santana, Sancha Helena de Lima Vale, Lucia Leite-Lais Arquivos De Neuro Psiquiatria, 2025 Amyotrophic lateral sclerosis (ALS) is a multifaceted neurodegenerative disorder with a poor prognosis. Weight loss and malnutrition emerge as significant clinical features during disease progression.To explore how demographic and clinical characteristics relate to survival in ALS patients, emphasizing the role of weight loss percentage at the time of diagnosis.We conducted a retrospective study that used the database of a multidisciplinary ALS care center in the city of Natal, Brazil.A total of 132 patients were included in the study. The mean age of the participants at symptom onset was of 56.9 years, and most of them were male (59.8%). Older age, bulbar onset, and faster disease progression were associated with weight loss ≥ 10% at diagnosis. Among 132 patients, 72% experienced death or tracheostomy, with a median survival of 34 months. Survival was notably reduced in patients aged ≥ 60 years, those with significant weight loss, rapid disease progression, or those submitted to gastrostomy. Weight loss and the rate of disease progression were the strongest predictors of reduced survival. Potential factors relating gastrostomy with reduced survival are discussed.The present study highlights the critical impact of weight loss and disease progression on survival in ALS patients, emphasizing the importance of early nutritional and clinical interventions. These findings underscore the need for comprehensive, multidisciplinary care strategies to address key prognostic factors and improve outcomes in ALS patients.
Effectiveness of a Learning Pathway on Food and Nutrition in Amyotrophic Lateral Sclerosis Karla Mônica Dantas Coutinho, Humberto Rabelo, Felipe Fernandes, Karilany Dantas Coutinho, Ricardo Alexsandro de Medeiros Valentim, Aline de Pinho Dias, Janaína Luana Rodrigues da Silva Valentim, Natalia Araújo do Nascimento Batista, Manoel Honorio Romão, Priscila Sanara da Cunha, Aliete Cunha-Oliveira, Susana Henriques, Luciana Protásio de Melo, Sancha Helena de Lima Vale, Lucia Leite-Lais, Kenio Costa de Lima Nutrients, 2025 Background/Objectives: Health education plays a vital role in training health professionals and caregivers, supporting both prevention and the promotion of self-care. In this context, technology serves as a valuable ally by enabling continuous and flexible learning. Among the various domains of health education, nutrition stands out as a key element in the management of Amyotrophic Lateral Sclerosis (ALS), helping to prevent malnutrition and enhance patient well-being. Accordingly, this study aimed to evaluate the effectiveness of the teaching and learning processes within a learning pathway focused on food and nutrition in the context of ALS. Methods: This study adopted a longitudinal, quantitative design. The learning pathway, titled “Food and Nutrition in ALS,” consisted of four self-paced and self-instructional Massive Open Online Courses (MOOCs), offered through the Virtual Learning Environment of the Brazilian Health System (AVASUS). Participants included health professionals, caregivers, and patients from all five regions of Brazil. Participants had the autonomy to complete the courses in any order, with no prerequisites for enrollment. Results: Out of 14,263 participants enrolled nationwide, 182 were included in this study after signing the Informed Consent Form. Of these, 142 (78%) completed at least one course and participated in the educational intervention. A significant increase in knowledge was observed, with mean pre-test scores rising from 7.3 (SD = 1.8) to 9.6 (SD = 0.9) on the post-test across all courses (p < 0.001). Conclusions: The self-instructional, technology-mediated continuing education model proved effective in improving participants’ knowledge about nutrition in ALS. Future studies should explore knowledge retention, behavior change, and the impact of such interventions on clinical outcomes, especially in multidisciplinary care settings.
Effect of Oral Zinc Supplementation on Phase Angle and Bioelectrical Impedance Vector Analysis in Duchenne Muscular Dystrophy: A Non-Randomized Clinical Trial Karina Marques Vermeulen-Serpa, Márcia Marilia Gomes Dantas Lopes, Camila Xavier Alves, Evellyn Camara Grilo, Thais Alves Cunha, Carolinne Thaisa de Oliveira Fernandes Miranda, Breno Gustavo Porfirio Bezerra, Lucia Leite-Lais, José Brandão-Neto, Sancha Helena de Lima Vale Nutrients, 2024 Zinc plays a crucial role in cell structure and functionality. Neurodegenerative Duchenne muscular dystrophy (DMD) alters muscle membrane structure, leading to a loss of muscle mass and strength. The objective of this study was to evaluate the changes in phase angle (PA) and bioelectrical impedance vector analysis (BIVA) results in patients with DMD after oral zinc supplementation. This clinical trial included 33 boys aged 5.6 to 24.5 years diagnosed with DMD. They were divided into three groups according to age (G1, G2, and G3) and supplemented with oral zinc. The mean serum zinc concentration was 74 μg/dL, and 29% of patients had concentrations below the reference value. The baseline values (mean (standard deviation)) of the bioelectrical impedance parameters PA, resistance (R), and reactance (Xc) were 2.59° (0.84°), 924.36 (212.31) Ω, and 39.64 (8.41) Ω, respectively. An increase in R and a decrease in PA and lean mass proportional to age were observed, along with a negative correlation (r = −0.614; p < 0.001) between age and PA. The average cell mass in G1 was greater than that in G3 (p = 0.012). There were no significant differences in serum zinc levels or bioelectrical impedance parameters before and after zinc supplementation. We conclude that this population is at risk of zinc deficiency and the proposed dosage of zinc supplementation was not sufficient to alter serum zinc levels, PA and BIVA results.
Educommunication in Nutrition and Neurodegenerative Diseases: A Scoping Review Karla Mônica Dantas Coutinho, Sancha Helena de Lima Vale, Manacés dos Santos Bezerril, Mônica Karina Santos Reis, Almudena Muñoz Gallego, Karilany Dantas Coutinho, Ricardo Valentim, Lucia Leite-Lais, Kenio Costa de Lima International Journal of Environmental Research and Public Health, 2024 Neurodegenerative diseases significantly impact individuals’ nutritional status. Therefore, nutritional education plays a crucial role in enhancing the understanding of food and nutrition, preventing or minimizing malnutrition, promoting well-being, and empowering patients and caregivers. Educommunication is a methodology that utilizes communication as a pedagogical tool, with the potential to positively enhance the teaching–learning process. This study aims to identify and map educommunication strategies designed to educate caregivers and patients with neurodegenerative diseases about food and nutrition. Methods: This scoping review followed the JBI Institute Reviewer’s Manual. The search was conducted between June 2022 and March 2023 in databases including PubMed/MEDLINE, Embase, Scopus, and Web of Science. Results: Out of 189 studies identified, 29 met the eligibility criteria, and only 3 were suitable for inclusion in this review. Conclusion: Studies using educommunication for food and nutrition education are scarce. Despite the limited number of studies included in this review, various educommunication strategies utilizing communication and information technologies were used. Educommunication strategies can facilitate knowledge acquisition in food and nutrition and change behaviors, resulting in health benefits for the participants. More studies on this subject are needed.
Effect of Zinc Supplementation on Body Composition of Duchenne Muscular Dystrophy Patients: A Quasi-Experimental Study Thais A. Cunha, Karina M. Vermeulen-Serpa, Evellyn C. Grilo, Mário E. T. Dourado-Júnior, Breno G. P. Bezerra, Núbia R. S. M. Torres, Márcia M. G. D. Lopes, Lucia Leite-Lais, José Brandão-Neto, Sancha H. L. Vale Journal of Nutrition and Metabolism, 2024 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.
Gender-specific bioelectrical impedance reference values in healthy children Karina Marques Vermeulen-Serpa, Márcia Marília Gomes Dantas Lopes, Carolinne Thaisa de Oliveira Fernandes Miranda, Camila Xavier Alves, Lucia Leite-Lais, José Brandão-Neto, Sancha Helena de Lima Vale Clinical Nutrition Open Science, 2023 Background & AimsBioimpedance parameters are important for assessing children's body composition and health status. However, interpreting these parameters is still challenging, especially without reference data and cut-off points.This study aimed to describe gender-specific reference phase angle (PhA) values and bioelectrical impedance vector analysis (BIVA) plots for healthy Brazilian children.MethodsChildren aged 6–9 years were recruited from four public schools in Natal, Brazil. Anthropometric measurements and bioelectrical impedance (BIA) were performed. The values of age, weight (kg), height (cm), resistance (Ω), and reactance (Ω) were measured. Only eutrophic children were included according to body mass index (BMI)-for-age Z-score. BIVA 2002 software was used for the construction of BIVA plots. A P-value < 0.05 was accepted as statistically significant.ResultsThe sample size was 108 children (boys, n= 49; girls, n= 59) with average age of 8.4 ± 0.77. The girls showed significantly higher values of resistance and resistance normalized by height (R/H) (P < 0.05) compared to the boys. Considering the 95% confidence ellipses for mean impedance vectors, there was no difference between boys and girls. Mean gender-specific reference values for PhA in our sample were traced. The 75th tertil for PhA were 5.79° and 5.40° for boys and girls, respectively. When comparing our sample with other ethnic groups was observed that we had higher values of R/H (582.4–620.7 versus 552.5–589.5) and lower values of Xc/H (53.8–54,7 versus 61.7–64,7) and PhA (5.3–5.1 versus 5.5–6.3) for boys and girls, respectively.ConclusionsThe PhA values and the new tolerance ellipses provided by our data reflect the normal growth and development of the prepubertal children studied. These reference values have significant clinical utility since they can point out altered body composition in other children due to pathophysiological conditions. Verifying changes in nutritional prognosis and hydration status allow early clinical and nutritional interventions.
A geographical study on amyotrophic lateral sclerosis in Rio Grande Do Norte, Brazil, from 2005 to 2018 Roberto Teodoro Gurgel De Oliveira, Glauciane Costa Santana, Maria De Jesus Gonçalves, Guilherme Augusto De Freitas Fregonezi, Sancha Helena De Lima Vale, Lucia Leite-Lais, Mário Emílio Teixeira Dourado Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2023 Objective: amyotrophic lateral sclerosis (ALS) is a rare and fatal neurodegenerative disorder with variable incidence and prevalence worldwide. However, clinical-epidemiological studies on ALS are scarce in Brazil. Thus, this study investigated whether ALS incidence had uniform spatial distribution in population-based cluster analysis in Rio Grande do Norte state (Brazil), from 2005 to 2018. Methods: new cases of ALS were identified in a database of the ALS multidisciplinary care center of the Onofre Lopes University Hospital in Natal (Rio Grande do Norte, Brazil). Approaches were based on incidence (empirical Bayes estimator and Moran's I analysis) and cluster analyses (Moran scatter plot and spatial correlogram). Results: a total of 177 patients (59% males) participated in the study; the mean age of ALS onset was 57 years. Mean annual incidence of ALS was 0.3769 per 100,000 inhabitants (95% confidence interval of 0.0889), higher in males than in females (0.4516 per 100,000 vs. 0.3044 per 100,000). According to spatial statistics, patients were homogeneously distributed throughout the studied area. Conclusion: a low estimate was observed compared with other populations. Results did not indicate areas of increased risk or significant spatial geographic dependence, suggesting a random ALS incidence in Rio Grande do Norte.
Nutritional therapy in amyotrophic lateral sclerosis: Protocol for a systematic review and meta-analysis Mariana Dantas de Carvalho Vilar, Karla Monica Dantas Coutinho, Sancha Helena de Lima Vale, Gidyenne Christine Bandeira Silva Medeiros, Grasiela Piuvezam, Lucia Leite-Lais, Jose Brandao-Neto BMJ Open, 2022 Introduction Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease characterised by the degeneration of motor neurons. Nutritional interventions in ALS are essential and must be based on scientific evidence to provide quality of healthcare, improve the quality of life and increase survival time. Therefore, this protocol of systematic reviews and meta-analyses aims to present a synthesis of evidence-based recommendations to support adequate nutrition therapy for patients with ALS. Methods and analysis The search will be performed using the following databases: PubMed, Excerpta Medica Database (Embase), Scopus, SciELO, Web of Science, LILACS, Cochrane Central Register of Controlled Trials (CENTRAL), ScienceDirect, ProQuest and Google Scholar. We will include clinical practice guidelines, treatment protocols, systematic reviews and clinical trials according to the three research questions to be answered related to nutrition therapy and interventions in patients with ALS. This protocol will be developed in accordance with the Preferred Reporting Items for Systematic Review and Meta-analysis Protocols. To evaluate the methodological quality of the studies, Appraisal of Guidelines, Research and Evaluation II, Cochrane Risk of Bias 2.0 and Risk of Bias In Non-randomized Studies of Interventions (ROBINS-I) tools will be used. In addition, the Grading of Recommendations Assessment, Development and Evaluation will be used to assess the quality of evidence and the strength of the recommendations. The findings will be summarised and presented descriptively according to the Cochrane Collaboration Handbook and the standard statistical meta-analysis techniques. Ethics and dissemination Ethical approval and human consent are not required because this is a protocol for systematic review and only secondary data will be used. Findings will be published in a peer-reviewed journal and presented at conferences. In case of any changes in this protocol, amendments will be updated in International Prospective Register of Systematic Reviews (PROSPERO) and the modifications will be explained in the final report of this review. PROSPERO registration number CRD42021233088.
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