Muscle Function Differences between Patients with Bulbar and Spinal Onset Amyotrophic Lateral Sclerosis. Does It Depend on Peripheral Glucose?

Jose Enrique De La Rubia Ortí,Jordi Caplliure-Llopis,Jose Luis Platero Armero,Esther Navarro-Illana,Jesús Escribá-Alepuz,María Benlloch,Alejandro Salazar,Sandra Sancho-Castillo,Claudia Emmanuela Sanchis-Sanchis,Carlos Barrios

doi:10.3390/jcm10081582

Abstract

Background: One of the pathogenic mechanisms of ALS disease is perturbed energy metabolism particularly glucose metabolism. Given the substantial difference in the severity and the prognosis of the disease, depending on whether it has a bulbar or spinal onset, the aim of the study was to determine metabolic differences between both types of ALS, as well as the possible relationship with muscle function. Materials and Methods: A descriptive, analytical, quantitative, and transversal study was carried out in hospitals and Primary Care centers in the region of Valencia, Spain. Fasting glucose and alkaline phosphatase (AP) levels in venous blood, muscle percentage, fat percentage, muscle strength (MRC scale), and functional capacity (Barthel Index) were measured in 31 patients diagnosed with ALS (20 with spinal onset ALS and 11 with bulbar onset ALS). A healthy control of 29 people was included. Results: No significant differences were observed in blood AP and glucose levels between spinal onset and bulbar onset ALS patients. However, a significant positive correlation was observed between the mean values of both substances in patients with spinal onset ALS. Moreover, a lower percentage of muscle mass and a higher percentage of fat mass were also seen in spinal ALS patients, who also presented lower muscle strength and lower functional capacity. Conclusion: The results of this study seem to point to a possible difference in the peripheral use of glucose between patients with bulbar onset ALS and spinal onset ALS, who appear to have possible insulin resistance. These metabolic differences could explain the lower muscle percentage and lower muscular function in spinal onset ALS patients, although further studies are required.

Highlights

Introduction distributed under the terms andAmyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease among those affecting motor neurons
There was no difference in sex, age, weight, body mass index (BMI), time since symptom onset, or functional capacity between them as determined by the ALS Functional Assessment Scale (ALSFRS)
When comparing disease progression using the King’s system in stage 1 or 2 ALS patients, there were no significant differences between both groups

Summary

Introduction

Introduction distributed under the terms andAmyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease among those affecting motor neurons. Despite being considered a rare disease, the prevalence of ALS has increased due to an incidence increase in the last few years [1] It is characterized by the selective death of motor neurons, and it can have a bulbar onset when conditions of the Creative Commons. It begins affecting the motor neurons in the medulla, or a spinal onset (limb onset) when it begins with a loss of strength and weakness in the extremities. Both types eventually lead to progressive paralysis of the voluntary muscles with progressive muscle waste, resulting in patient death [2] within 3 to 5 years [3]. A significant positive correlation was observed between the mean values of both substances in patients with spinal onset

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