ALSUntangled #63: ketogenic diets.

ALSUntangled reviews alternative and off label treatments with a goal of helping patients make more informed decisions about them. Here we review ketogenic diets. We shows that these have plausible mechanisms, including augmenting cellular energy balance and reducing excitotoxicity, neuroinflammation and oxidative stress. We review a mouse model study, anecdotal reports and trials in ALS and other diseases. We conclude that there is yet not enough data to recommend ketogenic diets for patients with ALS, especially in light of the many side effects these can have.

Fat-rich versus carbohydrate-rich nutrition in ALS: a randomised controlled study.

OBJECTIVE: There is growing evidence that the course of amyotrophic lateral sclerosis (ALS) may be influenced beneficially by applying high-caloric food supplements (HCSs). However, it is unknown which composition of nutrients offers optimal tolerability and weight gain. METHODS: We conducted a randomised controlled study (Safety and Tolerability of Ultra-high-caloric Food Supplements in Amyotrophic Lateral Sclerosis (ALS); TOLCAL-ALS study) in 64 patients with possible, probable or definite ALS according to El Escorial criteria. Patients were randomised into four groups: a high-caloric fatty supplement (HCFS; 405 kcal/day, 100% fat), an ultra-high-caloric fatty supplement (UHCFS; 810 kcal/day, 100% fat), an ultra-high-caloric, carbohydrate-rich supplement (UHCCS; 900 kcal/day, 49% carbohydrates) and an open control (OC) group without any supplement. The primary endpoint was tolerability. Patients were followed up over 4 weeks. RESULTS: Gastrointestinal side effects were most frequent in the UHCFS group (75.0%), while loss of appetite was most frequent in the UHCCS group (35.3%). During intervention, patients gained +0.9 kg/month of body weight (IQR -0.9 to 1.5; p=0.03) in the HCFS group and +0.9 kg/month (IQR -0.8 to 2.0; p=0.05) in the UHCFS group. A non-significant trend for weight gain (+0.6 kg/month (IQR -0.3 to 1.9; p=0.08)) was observed in the UHCCS group. Patients in OC group continued to lose body weight (-0.5 kg/month, IQR -1.4 to 1.3; p=0.42). INTERPRETATION: The findings suggest that HCSs frequently cause mild to moderate tolerability issues in patients with ALS, most notably gastrointestinal symptoms in high-fat supplements, and loss of appetite in high-carbohydrate supplements. All three HCSs tested are suited to increase body weight.

Palmitoylethanolamide: A Nutritional Approach to Keep Neuroinflammation within Physiological Boundaries-A Systematic Review.

Neuroinflammation is a physiological response aimed at maintaining the homodynamic balance and providing the body with the fundamental resource of adaptation to endogenous and exogenous stimuli. Although the response is initiated with protective purposes, the effect may be detrimental when not regulated. The physiological control of neuroinflammation is mainly achieved via regulatory mechanisms performed by particular cells of the immune system intimately associated with or within the nervous system and named "non-neuronal cells." In particular, mast cells (within the central nervous system and in the periphery) and microglia (at spinal and supraspinal level) are involved in this control, through a close functional relationship between them and neurons (either centrally, spinal, or peripherally located). Accordingly, neuroinflammation becomes a worsening factor in many disorders whenever the non-neuronal cell supervision is inadequate. It has been shown that the regulation of non-neuronal cells-and therefore the control of neuroinflammation-depends on the local "on demand" synthesis of the endogenous lipid amide Palmitoylethanolamide and related endocannabinoids. When the balance between synthesis and degradation of this bioactive lipid mediator is disrupted in favor of reduced synthesis and/or increased degradation, the behavior of non-neuronal cells may not be appropriately regulated and neuroinflammation exceeds the physiological boundaries. In these conditions, it has been demonstrated that the increase of endogenous Palmitoylethanolamide-either by decreasing its degradation or exogenous administration-is able to keep neuroinflammation within its physiological limits. In this review the large number of studies on the benefits derived from oral administration of micronized and highly bioavailable forms of Palmitoylethanolamide is discussed, with special reference to neuroinflammatory disorders.

Effect of High-Caloric Nutrition on Survival in Amyotrophic Lateral Sclerosis.

OBJECTIVE: Weight loss has been identified as a negative prognostic factor in amyotrophic lateral sclerosis, but there is no evidence regarding whether a high-caloric diet increases survival. Therefore, we sought to evaluate the efficacy of a high-caloric fatty diet (HCFD) for increasing survival. METHODS: A 1:1 randomized, placebo-controlled, parallel-group, double-blinded trial (LIPCAL-ALS study) was conducted between February 2015 and September 2018. Patients were followed up at 3, 6, 9, 12, 15, and 18 months after randomization. The study was performed at 12 sites of the clinical and scientific network of German motor neuron disease centers (ALS/MND-NET). Eligible patients were randomly assigned (1:1) to receive either HCFD (405kcal/day, 100% fat) or placebo in addition to riluzole (100mg/day). The primary endpoint was survival time, defined as time to death or time to study cutoff date. RESULTS: Two hundred one patients (80 female, 121 male, age = 62.4 ± 10.8 years) were included. The confirmatory analysis of the primary outcome survival showed a survival probability of 0.39 (95% confidence interval [CI] = 0.27-0.51) in the placebo group and 0.37 (95% CI = 0.25-0.49) in the HCFD group, both after 28 months (point in time of the last event). The hazard ratio was 0.97, 1-sided 97.5% CI = -∞ to 1.44, p = 0.44. INTERPRETATION: The results provide no evidence for a life-prolonging effect of HCFD for the whole amyotrophic lateral sclerosis population. However, post hoc analysis revealed a significant survival benefit for the subgroup of fast-progressing patients. ANN NEUROL 2020;87:206-216.

Nutritional counseling with or without mobile health technology: a randomized open-label standard-of-care-controlled trial in ALS.

BACKGROUND: Nutritional status is an important prognostic factor in Amyotrophic Lateral Sclerosis (ALS). We wished to study the safety, tolerability and efficacy of nutritional counseling with or without an mHealth application to maintain or increase body weight in ALS, compared to standard care. METHODS: In this randomized open-label, standard-of-care-controlled, single-center clinical trial, we randomly assigned adults with ALS to one of three nutritional interventions: counseling by their physician or nurse ("standard care"), counseling by a registered dietitian (RD) ("in-person"), or counseling supported by a mHealth app ("mHealth"). Both intervention arms received tailored nutrition recommendations and recorded dietary intake and weight biweekly (mHealth) or monthly (in-person). The primary outcome of weight and secondary and tertiary outcomes of calorie intake, ALSFRS-R, and quality of life (QOL) were recorded at each clinic visit and analyzed in an ITT mixed model analysis. RESULTS: A total of 88 participants were enrolled of whom 78 were included in this analysis. The three arms were well-balanced except for excess males in the mHealth arm and greater weight lost at baseline in the in-person arm. Participants in the mHealth arm increased their calorie intake at month 3 to an average of 94% (95% CI: 85, 103) of recommended calories, compared to 81% (95% CI: 72, 91, p = 0.06 vs. mHealth) in the standard care arm. After 6 months, calorie intake was not different among the three arms. Overall weight was stable across all three groups. QOL scores in the mHealth arm were stable over 3 months (0.3 points, 95% CI: - 1.7, 2.2) compared to worsening in standard care (- 2.1 points, 95% CI: - 4.0, - 0.2, p = 0.09 vs. mHealth), but all scores declined by 6 months. ALSFRS-R total scores declined by an average of - 2.6 points (95% CI: - 5.1, - 0.1) over 6 months in the mHealth arm (p = 0.13 vs. standard care) compared to - 5.8 points (95% CI: - 8.2, - 3.4, p = 0.74 vs. standard care) in the in-person and - 5.2 points (95% CI: - 7.6, - 2.9) in the standard care arm. CONCLUSIONS: Nutritional counseling by a registered dietitian (with or without support by an mHealth app) is safe but did not maintain weight significantly better than standard care in ALS patients. TRIAL REGISTRATION: https://clinicaltrials.gov/ identifier NCT02418546. Registered April 16, 2015.

Amyotrophic Lateral Sclerosis and Oxidative Stress: A Double-Blind Therapeutic Trial After Curcumin Supplementation.

OBJECTIVE: To investigate the efficacy of curcumin oral supplementation (600 mg/day, Brainoil), a natural antioxidant compound, in Amyotrophic Lateral Sclerosis (ALS). METHODS: Patients were randomized into two groups: Group A received placebo for 3 months, then Brainoil for the following 3 months, Group B took Brainoil for 6 months. The evaluations were conducted at basal (T0), after 3 months of double blinded Brainoil or placebo treatment (T1), and after the 3 month open-label phase (T2). Clinical evaluations and oxidative stress biomarkers, including oxidative protein products (AOPPs), ferric reducing ability (FRAP), total thiols (T-SH) and lactate, were evaluated, compared to a control group, during an incremental forearm exercise test. RESULTS: Over the entire study Group B showed a stable score of the ALS-FRS-r which decreased in Group A (p<0.01), in parallel with a reduction of AOPPs (p<0.01) which was not detected into Group A. Also FRAP exercise values remained stable in Group B, while in Group A they were reduced without treatment at T1 (0.05T0 exercise lactate was lower compared to Group A (p<0.01). Compared to controls, the whole ALS population showed a greater oxidative stress (p<0.001), those treated with curcumin (Group B) exhibiting decreased exercise AOPPs at T2 with values approaching those of controls. CONCLUSION: Although further studies are needed to confirm these data, treatment with curcumin shows encouraging results indicating a slight slowdown in disease progression, improving aerobic metabolism and oxidative damage, this also contributing to deepen knowledge into the pathogenic mechanisms of ALS.

Dietary intake and zinc status in amyotrophic lateral sclerosis patients / Ingesta dietética y estado de zinc en pacientes con esclerosis lateral amiotrófica

Background: There is considerable evidence that abnormal zinc homeostasis is related to amyotrophic lateral sclerosis (ALS) pathogenesis, and malnutrition is an independent prognostic factor for worsened survival of ALS patients. Objective: To evaluate the dietary intake and zinc status in patients with ALS, treated in a specialized outpatient facility in Natal, Brazil. Methods: Twenty patients with ALS (case group) and 37 healthy subjects (control group) were included. Clinical and anthropometric assessments were carried out and dietary intake was obtained from two 24-hour recalls. Plasma and urinary zinc concentrations were determined by atomic absorption spectrophotometry. Results: Most of the participants were eutrophic. Mean energy, protein, carbohydrate and fat intake was significantly lower for the case group. There was greater prevalence of inadequate zinc intake in the case group (35%) compared to controls (27%). Mean plasma zinc was significantly lower in the case group than in controls (77.13 ± 22.21 vs 87.84 ± 17.44 μgZn/dl). Urinary zinc did not differ significantly between cases and controls. In the case group, plasma and urinary zinc concentrations were below reference values in 50.0% and 52.6% of patients, respectively. Conclusion: A large portion of patients with ALS exhibited poor dietary intake and changes in body zinc status. The zinc deficiency found in half of the ALS patients may contribute to a worsened prognosis and should be the target of nutritional intervention that aims to correct this deficiency (AU)

Introducción: hay pruebas considerables de que los cambios en la homeostasis del zinc están relacionados con la patogénesis de la esclerosis lateral amiotrófica (ELA) y que la malnutrición es un factor pronóstico capaz de reducir la supervivencia de los pacientes con ELA. Objetivo: evaluar la ingesta dietética y el estado de zinc en pacientes con ELA, tratados en un centro de atención ambulatoria especializado en Natal, Brasil. Métodos: se incluyeron 20 pacientes con ELA (grupo de casos) y 37 sujetos sanos (grupo control). Se realizaron evaluaciones clínicas y antropométricas y se obtuvo la ingesta dietética en dos recordatorios de 24 horas. Las concentraciones plasmáticas y urinarias de zinc se determinaron por espectrofotometría de absorción atómica. Resultados: la mayoría de los participantes fueron eutróficos. El consumo medio de energía, proteínas, carbohidratos y grasas fue significativamente menor en el grupo de casos. Hubo una mayor prevalencia de ingesta inadecuada de zinc en el grupo de casos (35%) en comparación con los controles (27%). El zinc plasmático medio fue significativamente menor en el grupo de casos que en los controles (77,13 ± 22,21 frente a 87,84 ± 17,44 μgZn/dl). El zinc urinario no difirió significativamente entre los casos y los controles. En el grupo de casos, las concentraciones de zinc plasmático y urinario fueron inferiores a los valores de referencia en el 50,0% y 52,6% de los pacientes, respectivamente. Conclusión: gran parte de los pacientes con ELA exhibieron una ingesta dietética pobre y modificación en el estatus de zinc corporal. La deficiencia de zinc encontrada en la mitad de los pacientes con ELA puede contribuir a un empeoramiento del pronóstico y debe ser el objetivo de la intervención nutricional que apunta a corregir esta deficiencia (AU)

A Novel Iron Chelator-Radical Scavenger Ameliorates Motor Dysfunction and Improves Life Span and Mitochondrial Biogenesis in SOD1G93A ALS Mice.

The aim of the present study was to evaluate the therapeutic effect of the novel neuroprotective multitarget brain permeable monoamine oxidase inhibitor/iron chelating-radical scavenging drug, VAR10303 (VAR), co-administered with high-calorie/energy-supplemented diet (ced) in SOD1G93A transgenic amyotrophic lateral sclerosis (ALS) mice. Administration of VAR-ced was initiated after the appearance of disease symptoms (at day 88), as this regimen is comparable with the earliest time at which drug therapy could start in ALS patients. Using this rescue protocol, we demonstrated in the current study that VAR-ced treatment provided several beneficial effects in SOD1G93A mice, including improvement in motor performance, elevation of survival time, and attenuation of iron accumulation and motoneuron loss in the spinal cord. Moreover, VAR-ced treatment attenuated neuromuscular junction denervation and exerted a significant preservation of myofibril regular morphology, associated with a reduction in the expression levels of genes related to denervation and atrophy in the gastrocnemius (GNS) muscle in SOD1G93A mice. These effects were accompanied by upregulation of mitochondrial DNA and elevated activities of complexes I and II in the GNS muscle. We have also demonstrated that VAR-ced treatment upregulated the mitochondrial biogenesis master regulator, peroxisome proliferator-activated receptor-γ co-activator 1α (PGC-1α) and increased PGC-1α-targeted metabolic genes and proteins, such as, PPARγ, UCP1/3, NRF1/2, Tfam, and ERRα in GNS muscle. These results provide evidence of therapeutic potential of VAR-ced in SOD1G93A mice with underlying molecular mechanisms, further supporting the importance role of multitarget iron chelators in ALS treatment.

Alternative Fuels in Epilepsy and Amyotrophic Lateral Sclerosis.

This review summarises the recent findings on metabolic treatments for epilepsy and Amyotrophic Lateral Sclerosis (ALS) in honour of Professor Ursula Sonnewald. The metabolic impairments in rodent models of these disorders as well as affected patients are being discussed. In both epilepsy and ALS, there are defects in glucose uptake and reduced tricarboxylic acid (TCA) cycling, at least in part due to reduced amounts of C4 TCA cycle intermediates. In addition there are impairments in glycolysis in ALS. A reduction in glucose uptake can be addressed by providing the brain with alternative fuels, such as ketones or medium-chain triglycerides. As anaplerotic fuels, such as the triglyceride of heptanoate, triheptanoin, refill the TCA cycle C4/C5 intermediate pool that is deficient, they are ideal to boost TCA cycling and thus the oxidative metabolism of all fuels.

Assessment and nutrition education in patients with amyotrophic lateral sclerosis.

Neurological patients with amyotrophic lateral sclerosis (ALS)often deteriorate to a worsening nutritional status. The aim of this study was to compare the nutritional status and food intake after nutrition education in patients with ALS. Clinical, anthropometric and functional variables were analyzed. Fifty-three patients were monitored at an early stage of the disease. The average score on the functionality scale was 33 points. Initially only 3.8% were classified as low body weight. After three months, 50% showed significant variation in anthropometric measures related to muscle mass and body fat reserves without association with clinical variables. After nutritional guidance, there was an increase in the intake of all food groups, especially the dairy group (p <0.05).The change of the nutritional status occurs early in patients with amyotrophic lateral sclerosis, even in those previously eutrophic or over weight. There was an increase in food intake after nutritional guidance according to the food guide adapted to the Brazilian population.

Assessment and nutrition education in patients with amyotrophic lateral sclerosis / Avaliação e educação nutricional em pacientes com esclerose lateral amiotrófica

ABSTRACT Neurological patients with amyotrophic lateral sclerosis (ALS)often deteriorate to a worsening nutritional status. The aim of this study was to compare the nutritional status and food intake after nutrition education in patients with ALS. Clinical, anthropometric and functional variables were analyzed. Fifty-three patients were monitored at an early stage of the disease. The average score on the functionality scale was 33 points. Initially only 3.8% were classified as low body weight. After three months, 50% showed significant variation in anthropometric measures related to muscle mass and body fat reserves without association with clinical variables. After nutritional guidance, there was an increase in the intake of all food groups, especially the dairy group (p <0.05).The change of the nutritional status occurs early in patients with amyotrophic lateral sclerosis, even in those previously eutrophic or over weight. There was an increase in food intake after nutritional guidance according to the food guide adapted to the Brazilian population.

RESUMO Pacientes neurológicos com esclerose lateral amiotrófica frequentemente evoluem com piora do estado nutricional. O objetivo desse estudo foi comparar o estado nutricional e a ingestão alimentar depois da orientação nutricional em pacientes com ELA. Variáveis clínicas,antropométricas e funcionais foram analisadas. 53 pacientes foram avaliados na fase inicial da doença. A pontuação média da escala de funcionalidade foi de 33 pontos. Inicialmente apenas 3,8% foram classificados como baixo peso. Após três meses, 50% apresentaram variação significativa nas medidas antropométricas relacionadas com reservas de massa muscular e gordura corporal, sem associação com variáveis clínicas. Após orientação nutricional, houve um aumento na ingestão de alimentos de todos os grupos com relevância para o grupo de lacticínios (p <0,05). A mudança do estado nutricional ocorre precocemente em pacientes com ELA, mesmo naqueles anteriormente eutróficos ou sobrepeso. Houve um aumento na ingestão de alimentos após orientação nutricional de acordo com o guia alimentar adaptado da população brasileira.

Association Between Dietary Intake and Function in Amyotrophic Lateral Sclerosis.

IMPORTANCE: There is growing interest in the role of nutrition in the pathogenesis and progression of amyotrophic lateral sclerosis (ALS). OBJECTIVE: To evaluate the associations between nutrients, individually and in groups, and ALS function and respiratory function at diagnosis. DESIGN, SETTING, AND PARTICIPANTS: A cross-sectional baseline analysis of the Amyotrophic Lateral Sclerosis Multicenter Cohort Study of Oxidative Stress study was conducted from March 14, 2008, to February 27, 2013, at 16 ALS clinics throughout the United States among 302 patients with ALS symptom duration of 18 months or less. EXPOSURES: Nutrient intake, measured using a modified Block Food Frequency Questionnaire (FFQ). MAIN OUTCOMES AND MEASURES: Amyotrophic lateral sclerosis function, measured using the ALS Functional Rating Scale-Revised (ALSFRS-R), and respiratory function, measured using percentage of predicted forced vital capacity (FVC). RESULTS: Baseline data were available on 302 patients with ALS (median age, 63.2 years [interquartile range, 55.5-68.0 years]; 178 men and 124 women). Regression analysis of nutrients found that higher intakes of antioxidants and carotenes from vegetables were associated with higher ALSFRS-R scores or percentage FVC. Empirically weighted indices using the weighted quantile sum regression method of "good" micronutrients and "good" food groups were positively associated with ALSFRS-R scores (ß [SE], 2.7 [0.69] and 2.9 [0.9], respectively) and percentage FVC (ß [SE], 12.1 [2.8] and 11.5 [3.4], respectively) (all P < .001). Positive and significant associations with ALSFRS-R scores (ß [SE], 1.5 [0.61]; P = .02) and percentage FVC (ß [SE], 5.2 [2.2]; P = .02) for selected vitamins were found in exploratory analyses. CONCLUSIONS AND RELEVANCE: Antioxidants, carotenes, fruits, and vegetables were associated with higher ALS function at baseline by regression of nutrient indices and weighted quantile sum regression analysis. We also demonstrated the usefulness of the weighted quantile sum regression method in the evaluation of diet. Those responsible for nutritional care of the patient with ALS should consider promoting fruit and vegetable intake since they are high in antioxidants and carotenes.

Enteral feeding through endoscopic gastrostomy in amyotrophic lateral sclerosis patients / Nutrición enteral por gastrostomía endoscópica en pacientes con esclerosis lateral amiotrófica

Background: Dysphagia is common in amyotrophic lateral sclerosis (ALS) and may result in malnutrition. Endoscopic gastrostomy (PEG) is recommended when oral feeding is unsafe. This work aims to assess the effectiveness and safety of PEG feeding on improving nutritional and prognostic parameters in ALS patients. Methods: Observational and retrospective study using records from ALS patients referred for gastrostomy. Age, gender and mortality data were collected. NRS 2002, body mass index (BMI), serum albumin, transferrin and total cholesterol were recorded at the time of PEG (T0) and repeated after 3 months (T3). The evolution of these parameters was analysed and compared to survival. Results: Data from 37 ALS patients (18 men/19 women) aged 43-88 years (mean: 69 years). All patients presented NRS 2002 ≥ 3 points. On average, patients underwent gastrostomy 11 months after diagnosis. No major procedural complications occurred. Mean survival after diagnosis was 22.2 months. Mortality rate at 3 months was 21.6% with a mean survival after PEG of 11.2 months. Albumin, transferrin and cholesterol levels increased from T0 to T3 without reaching statistical significance. Higher albumin (R = 0.3) and transferrin (R = 0.4) at admission tend to be positively correlated with survival. Mean BMI was similar at the two moments but higher initial values were associated with better outcome (R2 = 0.39, p < 0.05). Conclusions: PEG is a safe and effective technique for enteral feeding and should be considered early in ALS patients with dysphagia. Higher BMI predicts longer survival. The association between higher serum proteins and survival must be confirmed in further studies (AU)

Introducción: la disfagia es común en pacientes con esclerosis lateral amiotrófica (ELA) y pude resultar en desnutrición. Se recomienda la gastrostomía endoscópica (PEG) cuando la alimentación oral no es más segura. Este trabajo tiene como objetivo evaluar la eficacia y seguridad de la alimentación por PEG en la mejora de los parámetros nutricionales y de pronóstico en pacientes con ELA. Métodos: estudio observacional y retrospectivo que utilizó los registros clínicos de pacientes con ELA referidos para gastrostomía. Se recogieron datos sobre la edad, el género y la mortalidad. Se registró el NRS 2002, el índice de masa corporal (IMC), la albúmina sérica, la transferrina y el colesterol total en el momento de PEG (T0) y después de 3 meses (T3). Resultados: fueron recolectados los datos de 37 pacientes con ELA (18 hombres/19 mujeres), con edades comprendidas entre 43-88 años (media: 69 años). Todos los pacientes presentaron NRS 2002 ≥ 3 puntos. En promedio, los pacientes fueron sometidos a gastrostomía 11 meses después del diagnóstico. No hubo complicaciones mayores del procedimiento. La media de supervivencia después del diagnóstico de ELA fue de 22,2 meses. La tasa de mortalidad a los 3 meses fue de 21,6%, con una supervivencia media de 1,.2 meses después de PEG. Los niveles de albúmina, transferrina y colesterol aumentaron de T0 a T3, sin embargo sin alcanzar significación estadística. Mayores niveles de albúmina (R = 0,3) y de transferrina séricas (R = 0,4) en el momento de PEG tienden a estar correlacionados positivamente con una supervivencia más larga. El IMC medio fue similar en los dos momentos, pero valores iniciales más altos están asociados con un mejor pronóstico (R2 = 0,39, p < 0,05). Conclusiones: PEG es una técnica segura y efi caz para la nutrición enteral y se debe considerar temprano en pacientes con ELA y disfagia. Un mayor IMC predice una mayor supervivencia. La asociación entre proteínas séricas más altas y la supervivencia debe ser confirmada en estudios posteriores (AU)

The influence of the initial state of nutrition on the lifespan of patients with amyotrophic lateral sclerosis (ALS) during home enteral nutrition / La influencia del estado inicial de la nutrición en la esperanza de vida de pacientes con esclerosis lateral amiotrófica (ALS) durante la nutrición enteral en casa

Aim: Amyotrophic lateral sclerosis (ALS) is a chronic, neurodegenerative disease, which leads to development of malnutrition. The main purpose of this research was to analyze the impact of malnutrition on the course of the disease and long-term survival. Material and methods: A retrospective analysis has been performed on 48 patients (22 F [45,83%] and 26 M [54,17%], the average age of patients: 66,2 [43-83]) in 2008-2014.The analysis of the initial state of nutrition was measured by body mass index (BMI), nutritional status according to NRS 2002, SGA and concentration of albumin in blood serum. Patients were divided into two groups, depending on the state of nutrition: well-nourished and malnourished. The groups were created separately for each of these, which allowed an additional comparative analysis of techniques used for the assessment of nutritional status. Results: Proper state of nutrition was interrelated with longer survival (SGA: 456 vs. 679 days, NRS: 312 vs. 659 vs. 835 days, BMI: respectively, 411, 541, 631 days, results were statistically significant for NRS and BMI). Concentration of albumin was not a prognostic factor, but longer survival was observed when level of albumin was increased during nutritional therapy. Conclusions: The initial nutrition state and positive response to enteral feeding is associated with better survival among patients with ALS. For this reason, nutritional therapy should be introduced as soon as possible (AU)

Objetivo: la esclerosis lateral amiotrófica (ELA) es una enfermedad crónica, neurodegenerativa que genera malnutrición. El objetivo principal de este estudio es analizar el impacto de la malnutrición en el curso de la enfermedad y la sobrevida a largo plazo. Materiales y métodos: se realizó un análisis retrospectivo de 48 pacientes (22 mujeres [45.83%] y 26 hombres [54.17%], con un promedio de edad de 66,2 [43-83]) del 2008 al 2014. El análisis del estado nutricional inicial se midió utilizando el índice de masa corporal (IMC), el estado nutricional de acuerdo al NRS 2002, la valoración global subjetiva (VGS) y la concentración sérica de albúmina. Los pacientes se dividieron en dos grupos, dependiendo del estado nutricional en el que se encontraban: bien nutridos o malnutridos. Los grupos se crearon por separado, lo que permitió un análisis comparativo adicional de las técnicas utilizadas para la evaluación del estado nutricional. Resultados: un estado nutricional adecuado se relaciona con mayor sobreviva a largo plazo (456 vs. 679 días, NRS 312 vs. 659 vs. 835 días, IMC respectivamente: 41, 541, 631 días, resultados estadísticamente significativos para NRS e IMC). Las concentraciones de albúmina no fueron un factor pronóstico, pero se observó mayor sobrevida si las concentraciones de albúmina incrementaban durante el tratamiento nutricional. Conclusiones: el estado nutricional inicial y la respuesta positiva a la alimentación enteral están asociados a mayor sobrevida en pacientes con ELA, razón por la cual, la terapia nutricional debe de iniciarse lo antes posible (AU)

Atención clínica, toma de decisiones y soporte nutricional en pacientes con esclerosis lateral amiotrófica / Clinical care, decision making and nutritional support in patients with amyotrophic lateral sclerosis

Introducción: En los pacientes afectos de Esclerosis Lateral Amiotrófica, es indispensable ofertar una adecuada información, que conlleve una acertada toma de decisiones, debido a las situaciones de alta complejidad que presentan durante su enfermedad. Con esta idea de fondo, se creó en nuestro Hospital Universitario Puerta del Mar de Cádiz un equipo interdisciplinar e interniveles para la atención de pacientes con esta entidad. Objetivos: Comparar variables relacionadas con la evolución clínica y la de toma de decisiones antes y después de la creación del equipo. Material y Método: Estudio descriptivo, observacional. Se comparan variables, pre y post intervención, destacando: sintomatología que motiva la consulta o el ingreso, episodios de atención en el hospital, interconsultas a profesionales, registro en la historia clínica de haber proporcionado información sobre la posibilidad de expresar la Voluntad Vital Anticipada y/o la existencia de la misma, soporte nutricional recibido. Resultados: Se obtuvieron 15 pacientes en el primer grupo y 13 en el segundo. Existe mejora en los porcentajes obtenidos en todas las variables, destacando el registro de voluntades vitales, toma de decisiones y soporte nutricional. Discusión-Conclusiones: La creación de un equipo interdisciplinar que hace partícipe al paciente de todo su proceso, además de asegurar una adecuada toma de decisiones por parte del paciente, conlleva la mejora de los resultados de indicadores clínicos (AU)

Background: In cases of ALS, due to the highly complex situations arising during the disease, it is essential to provide adequate information to the patient, so they can make suitable decisions concerning quality of life and symptom management over the course of their disease. To this end, an interdisciplinary and interlevel team has been created in the Hospital Universitario Puerta del Mar of Cádiz to provide the best care for people living with ALS. Objectives: Compare variables related to the clinical outcome and decision in-making before and after the creation of the team. Methods: Descriptive and observational study. Variables under evaluation were compared pre- and post- intervention, including: symptoms at the admission or consultation stages, care taking episodes in the hospital, interdepartmental clinical issues, entries in the medical record of existence or prospect of completing the advance health care directive, decision-making and nutritional support. Result: For sake of comparison, two groups were formed, the first one was made of 15, whereas the second one composed of 13 patients. There was a significant improvement in all the variables under study. It is especially remarkable the advance in the living will records, decision-making and nutritional support. Discussion-Conclusion: The creation of an interdisciplinary team capable fo engaging the patient of ALS in the clinical issues and symptom management over the course of the disease, not only ensures an adequate decision-making by the patient, but improves the results of the corresponding clinical indicators (AU)

La opinión del paciente cuenta: Experiencia en la atención nutricional en un equipo multidisciplinar de ELA / The patient’s opinion matters: experience in the nutritional care in an ALS multidisciplinary team

La Esclerosis Lateral Amiotrófica (ELA) es una enfermedad neurodegenerativa que carece de tratamiento curativo por lo que el tratamiento será sintomático en Unidades Multidisciplinares. Éstas estarán compuestas por los profesionales expertos necesarios para la atención del enfermo con una visión interdisciplinar que actúe de una forma coordinada ante las diferentes situaciones que puedan surgir a lo largo de la evolución de la enfermedad. En este sentido existen diferentes estudios que demuestran una mayor supervivencia en los pacientes tratados en el marco de un equipo multidisciplinar comparado con el tratamiento por especialidades aisladas. En el Hospital Universitario de Bellvitge desde 2004 se ha creado una Unidad Multidisciplinar de ELA que integra neurólogo, neumólogo, nutricionista, endocrinólogo, rehabilitador, fisioterapeuta, psicólogo, trabajador social, enfermera gestora, logopeda, administrativo. Para evaluar el impacto de la atención multidisciplinar de nuestro programa se evaluaron 418 pacientes diagnosticados de ELA, 84 pacientes habían recibido atención por consultas de neurología general y 334 tratados bajo un modelo de atención multidisciplinar. Los pacientes que fueron tratados en la unidad de atención multidisciplinar tuvieron una mediana de supervivencia de 1246 días (IC 1109 - 1382), 104 días superior a la mediana de 1148 días de aquellos seguidos en la consulta de neurología general (IC 998 - 1297). Esta diferencia fue estadísticamente significativa (log-rank10,8, p 0,008). Este beneficio fue independiente del hecho de haber recibido tratamiento con riluzole, ventilación mecánica no invasiva o gastrostomía percutánea. La valoración nutricional se realizará desde la primera visita y en todos los controles posteriores. Es importante realizar las medidas antropométricas y detectar la pérdida de peso involuntaria y las posibles causas desencadenantes para establecer el tratamiento nutricional adecuado. La exploración de la disfagia permite establecer el consejo dietético adecuado, la introducción de espesantes para adecuar la textura de los alimentos o la suplementación nutricional con fórmulas hipercalóricas para evitar o corregir la pérdida de peso. En el caso que estas medidas no sean suficientes o bien existe el riesgo de fracaso de la función respiratoria se indicará la colocación precoz de gastrostomía. El análisis de 140 pacientes con ELA (92 controles y 48 con gastrostomía radiológica percutánea), no mostró diferencias en el tiempo de supervivencia media entre los grupos (32 vs 33,9 meses, log Rank 1,86 p= 0,39). Ningún paciente presentó complicaciones mayores. A pesar de no evidenciar cambios en la supervivencia, el uso de la gastrostomía debe ser entendido como un tratamiento destinado a mejorar la calidad de vida y el bienestar del paciente. El apoyo psicosocial a la persona y al entorno familiar es fundamental para poder integrar todos los cambios y situaciones que surgirán en la evolución de la enfermedad. Debe iniciarse desde el diagnóstico ya que la atención temprana contribuye a su mejor formación, prevenir situaciones de deterioro y afrontar el proceso de dependencia. Además existe la posibilidad de utilizar la tecnología y las redes sociales para complementar el modelo asistencial clásico. En el caso concreto de la Unidad Funcional de ELA del HUB, las personas afectadas y sus familias cuentan con los recursos del Aula Paciente y el blog ELA, creados con el objetivo de ofrecer espacios de diálogo entre pacientes, familiares y cuidadores. El grado de satisfacción del paciente con la atención recibida por la Unidad Multidisciplinar de ELA sobre la accesibilidad al servicio, la información recibida y la calidad de la atención, globalmente fue valorada cómo buena en el 52,8% o muy buena en el 29,2 % de los pacientes. Conclusión: La atención a la persona afectada de ELA debe ser contemplada en el marco de un equipo multidisciplinar integrado por todos los profesionales que vayan a intervenir a lo largo del proceso de su enfermedad para ofrecer una mayor supervivencia con la mejor atención y calidad de vida (AU)

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Ketogenic diet in neuromuscular and neurodegenerative diseases.

An increasing number of data demonstrate the utility of ketogenic diets in a variety of metabolic diseases as obesity, metabolic syndrome, and diabetes. In regard to neurological disorders, ketogenic diet is recognized as an effective treatment for pharmacoresistant epilepsy but emerging data suggests that ketogenic diet could be also useful in amyotrophic lateral sclerosis, Alzheimer, Parkinson's disease, and some mitochondriopathies. Although these diseases have different pathogenesis and features, there are some common mechanisms that could explain the effects of ketogenic diets. These mechanisms are to provide an efficient source of energy for the treatment of certain types of neurodegenerative diseases characterized by focal brain hypometabolism; to decrease the oxidative damage associated with various kinds of metabolic stress; to increase the mitochondrial biogenesis pathways; and to take advantage of the capacity of ketones to bypass the defect in complex I activity implicated in some neurological diseases. These mechanisms will be discussed in this review.