El médico de familia ante la Esclerosis Lateral Amiotrófica

La esclerosis lateral amiotrófica (ELA) es una enfermedad neurodegenerativa con una incidencia de 1-2 casos por 100.000 habitantes/año y una prevalencia de 4-6 casos por 100.000 habitantes (3,5 casos por 100.000 habitantes en España). La mayoría de los casos de ELA son esporádicos, pero el 5-10 % son familiares con herencia autosómica dominante. La edad media de aparición para ELA esporádica es de alrededor de los 60 años. El objetivo del presente estudio de casos ha sido analizar la importancia del papel del médico de atención primaria en la sospecha diagnóstica, derivación, seguimiento y acompañamiento del paciente con ELA y su familia. Para ello se han analizado dos casos clínicos de los tipos más característicos de ELA y las actuaciones de los médicos de atención primaria que los han atendido, reflejando que una mayor difusión de los síntomas y signos de ELA podrían contribuir a un diagnóstico más precoz y con mayor grado de certeza.

The influence of the initial state of nutrition on the lifespan of patients with amyotrophic lateral sclerosis (ALS) during home enteral nutrition

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.