Abstract

Objective: Musculoskeletal functional deterioration in Amyotrophic lateral sclerosis (ALS) is associated with an increase in bone fractures. The purpose of this study was to evaluate the influence of sex, ALS type, on bone quality in patients with ALS compared to healthy controls. The impact on bone health of the clinical status and some metabolic parameters was also analyzed in ALS patients.Methods: A series of 33 voluntary patients with ALS, and 66 healthy individuals matched in sex and age underwent assessment of bone mass quality using quantitative ultrasound (QUS) of the calcaneus. Ultrasonic broadband attenuation (BUA), the speed of sound (SOS), stiffness index and T-score were measured. Bone mineral density (BMD) was estimated using standard equations. Apart from fat and muscle mass percentage determinations, clinical baseline measures in ALS patients included ALSFRS-R score, Barthel index for activities of daily living, pulmonary function measured using FVC, and muscular strength assessed by a modified MRC grading scale. Laboratory tests included serum calcium, 25-HO-cholecalciferol (Vitamin D), alkaline phosphatase (ALP), T4 and TSH.Results: All bone parameters evaluated were statistically significant lower in ALS patients than in healthy controls. ALS females showed significantly lower bone parameters than healthy females. According to the estimated BMD, there were 25 ALS patients (75.8%) and 36 (54.5%) healthy individuals showing an osteoporotic profile (BMD <0.700 g/cm2). Only 16.7% of the ALS females had T-scores indicative of healthy bones. There was no correlation between any of the clinical parameters analyzed and the bone QUS measurements. Vitamin D and TSH levels positively correlated with all the bone parameters.Conclusions: This study confirms that ALS patients, particularly females, exhibited deteriorated bone health as compared to healthy individuals. These structural bone changes were independent of ALS subtype and clinical status. Bone health in ALS patients seems to be related to certain metabolic parameters such as Vitamin D and TSH levels.

Highlights

  • Amyotrophic lateral sclerosis (ALS) is a devasting neurodegenerative disease with progressive motor neuron damage leading to the death of the patients in a very short average time, from 16 months to 3 years after diagnosis [1]

  • The total population that was included in the study was 99 individuals (33 ALS patients and 66 healthy controls)

  • Age and the general anthropometric characteristics of the series are shown in Table 1 including date discriminated by sex

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Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is a devasting neurodegenerative disease with progressive motor neuron damage leading to the death of the patients in a very short average time, from 16 months to 3 years after diagnosis [1]. From a clinical point of view, two main types of ALS are distinguished: bulbar and medullary, depending on whether the upper or lower motor neuron, respectively, is initially affected. In both cases, a series of characteristic signs and symptoms appear, reflecting progressive musculoskeletal deterioration. Musculoskeletal functional deterioration in ALS is associated with an increase in bone fractures, due to a decrease in bone density [6,7,8]

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