Weerkamp, P. M. M., Mol, E. M., Sweere, D. J. J., Schrans, D. G. M., Vermeulen, R. J., Klinkenberg, S., Hurks, P. P. M., & Hendriksen, J. G. M. (2022). Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
Brain sciences, 12(11), 1544. https://doi.org/10.3390/brainsci12111544
Dystrophinopathies are a group of genetic disorders that primarily affect males and are characterized by progressive muscle weakness and degeneration. These conditions include Duchenne muscular dystrophy (DMD), which is the most common and severe type of dystrophinopathy, and Becker muscular dystrophy (BMD), which has a more heterogenous phenotype. In addition to physical symptoms, individuals with dystrophinopathies may also experience cognitive impairments, such as difficulties with attention, memory, and learning.
A recent research paper titled “Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis” aimed to investigate intelligence in males with dystrophinopathies using a specific type of intelligence test called the Wechsler scale. In research and clinical practice, Wechsler Intelligence Scales are internationally the most frequently used tests to determine intelligence. The main reason to focus on Wechsler Intelligence Scales alone is to ensure consistency of test scores and therefore minimalising variation of methods/instruments used. To conduct or study, we performed a systematic review and meta-analysis, which involved analysing data from multiple previous studies that examined Wechsler intelligence test scores in males with dystrophinopathies. By pooling the data from these studies, we were able to obtain a larger sample size and gain more robust insights into the intelligence profile of individuals with dystrophinopathies.
In this study, 43 studies were included, reporting data on 1472 males with dystrophinopathies; with FSIQ scores available for 1234 DMD (k = 32) and 101 BMD (k = 7). The findings of the meta-analysis revealed that males with dystrophinopathies generally exhibited lower scores on the Wechsler intelligence tests compared to the general population. DMD males score, on average, one standard deviation below average (FSIQ = 84.76) and significantly lower than BMD (FSIQ = 92.11). The results of this study have important implications for individuals with dystrophinopathies, their families, and healthcare providers. By highlighting the cognitive challenges faced by these individuals, the study underscores the need for comprehensive assessments that consider both physical and cognitive aspects of their health. Such assessments can help guide the development of targeted interventions and educational strategies to optimize the quality of life and support the cognitive development of individuals with dystrophinopathies.
In summary, we recommend to routinely offer intelligence and cognitive functioning screening to patients with DMD and BMD and that the Wechsler Intelligence scales provide a solid instrument to measure intelligence in individuals with dystrophinopathies. These data contribute to our understanding of the unique challenges faced by individuals with dystrophinopathies and may contribute to help patients achieve optimal participation in society (e.g., education, work, daily functioning).