Saoudi, A., Zarrouki, F., Sebrié, C., Izabelle, C., Goyenvalle, A., & Vaillend, C. (2021). Emotional behavior and brain anatomy of the mdx52 mouse model of Duchenne muscular dystrophy

Disease models & mechanisms, 14(9), dmm049028.

Lay Summary

Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by muscle weakness and degeneration. While the primary symptoms of DMD are related to muscle problems, DMD also affects the brain, leading to cognitive, emotional and behavioural problems. Understanding the impact of DMD on the brain is crucial for developing comprehensive treatment approaches.  

The paper titled “Emotional Behavior and Brain Anatomy of the mdx52 Mouse Model of Duchenne Muscular Dystrophy” investigates the emotional behaviour and brain anatomy in a mouse model of DMD called mdx52. This model mimics many of the features observed in individuals with DMD, providing insights into the neurological aspects of the condition.  

The researchers in this study examined the emotional behaviours of the mdx52 mice using various tests assessing anxiety-like behaviours, exploration and emotional memories in mice. Additionally, the researchers examined the brain anatomy of the mdx52 mice using magnetic resonance imaging (MRI).  

The findings of the study demonstrated that the mdx52 mice exhibited increased anxiety-like behaviour, reduced exploration, exacerbated stress reactivity and difficulties to learn and remember emotion-related events. These findings suggest that DMD not only affects muscle function but also has implications for regulating emotional behaviours.  The MRI analysis did not detect any structural differences in the brains of the mdx52 mice compared to controls. This suggests that the observed emotional and behavioural changes in the mdx52 mice cannot be attributed to gross abnormalities of brain development.  

By investigating the emotional behaviour and brain anatomy of the mdx52 mouse model, this study provides valuable insights into the neurological aspects of DMD. The findings suggest that DMD has a broader impact beyond the muscles, particularly affecting emotional regulation.  

Understanding the emotional and behavioural consequences of DMD is essential for improving the quality of life for individuals with the condition. These insights may guide the development of interventions aimed at addressing the emotional challenges faced by individuals with DMD, potentially leading to more holistic and comprehensive treatment approaches.  

It is important to note that the mdx52 mouse model may not fully represent the complexities of DMD in humans, and further research is needed to confirm these findings in human patients. Nevertheless, this study contributes to our understanding of the neurological manifestations of DMD and highlights the need for a multidisciplinary approach to address the physical, emotional, and cognitive aspects of the condition.