Systemic Inflammation Lowers With Decreased Muscle Function in Duchenne Muscular Dystrophy, According to Study

The causes behind Duchenne muscular dystrophy (DMD) are not fully understood and, as in most muscular dystrophies, no current cure or specific treatment exists. As such, management of affected patients involves corticoids and is directed to the complications of the disease, namely physical therapy for muscle weakness and non-invasive ventilation in case of respiratory problems. A new study entitled “Systemic Inflammation in Duchenne Muscular Dystrophy: Association with Muscle Function and Nutritional Status” recently published in the BioMed Research International journal tries to dig deeper into the understanding of the disease pathological processes by establishing a link between inflammation and muscle function and/or nutritional status (underweight, normal weight, and overweight/obese) on these patients.

DMD is the most frequent genetically inherited human myopathy (i.e, disease of the muscles) and, as the name implies, is characterized by wasting of the muscles throughout the body. The disease is caused by a mutation of the dystrophin gene at the X chromosome and is characterized by muscle weakness of the voluntary muscles. The hips, pelvic area, thighs, shoulders and calves are usually the first affected areas. While both sexes can carry the mutation, females are rarely affected as they have two copies of the X chromosome – one of which is normal in healthy carriers – whereas males have only one.

In this study, a team of researchers from the Centro Medico Nacional Siglo XXI, Mexico, evaluated several inflammatory substances in blood, body mass index and muscle function using a clinically meaningful scale (Vignos Scale) in 66 patients suffering from DMD. The results showed that patients with better muscle function had the highest concentration of inflammatory substances (CK, IL-1 and TNF alpha). Moreover, nutrition level did not affect the inflammatory status.

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The data demonstrated that patients confined to wheelchair or to bed (i.e., most affected muscle function) had the lowest inflammatory status which could be due to muscle loss in those patients. Conversely, in the early stages of the disease, when patients have better muscular function, the inflammation is increased. Although more studies are needed, in the future these substances (CK, IL-1 and TNF alpha) could be used as markers for disease activity to help physicians better tackle the disease.