Neurotrophins, cytokines, oxidative parameters and funcionality in Progressive Muscular Dystrophies.

Clarissa M Comim,Flávio Kapczinski,Andreza Hoepers,Felipe Dal-Pizzol,Lisiane Tuon,Gisiane B Mathia,Maria I Rosa,João Quevedo

doi:10.1590/0001-3765201520140508

Abstract

We investigated the levels of brain derived-neurotrophic factor (BDNF), cytokines and oxidative parameters in serum and tried to correlate them with the age and functionality of patients with Progressive Muscle Dystrophies (PMD). The patients were separated into six groups (case and controls pared by age and gender), as follows: Duchenne Muscular Dystrophy (DMD); Steinert Myotonic Dystrophy (SMD); and Limb-girdle Muscular Dystrophy type-2A (LGMD2A). DMD patients (± 17.9 years old) had a decrease of functionality, an increase in the IL-1β and TNF-α levels and a decrease of IL-10 levels and superoxide dismutase activity in serum. SMD patients (± 25.8 years old) had a decrease of BDNF and IL-10 levels and superoxide dismutase activity and an increase of IL-1β levels in serum. LGMD2A patients (± 27.7 years old) had an decrease only in serum levels of IL-10. This research showed the first evidence of BDNF involvement in the SMD patients and a possible unbalance between pro-inflammatory and anti-inflammatory cytokine levels, along with decreased superoxide dismutase activity in serum of DMD and SMD patients.

Highlights

Progressive Muscular Dystrophies (PMD) are a heterogeneous group of disorders that primarily affect skeletal muscles
Duchenne Muscular Dystrophy (DMD) patients presented a decline in the functionality of those with the mean age of 17.9 years old
Late-onset muscular dystrophies, as Steinert Myotonic Dystrophy (SMD) and limb-girdle muscular dystrophy type 2A (LGMD2A), may be mild and associated with slight weakness or fatigability induced by effort, without significant loss of functionality (Chelly and Desguerre 2013)

Summary

Introduction

Progressive Muscular Dystrophies (PMD) are a heterogeneous group of disorders that primarily affect skeletal muscles. It is characterized by Duchenne Muscular Dystrophy (DMD) is the most severe form of PMD worldwide. It is a fatal recessive X-linked muscular disease affecting about 1 in 3500 live-born human males. This weakness is due to an irreversible ongoing degeneration of skeletal muscle and results in a loss of mobility at the beginning of the second decade and a fatal outcome in the third decade. The LGMD2A are caused by primary mutations in CAPN3 gene (Guglieri et al 2005)

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