Background: Dystrophinopathies comprise a spectrum of X-linked progressive neuromuscular disorders caused by mutations in DMD gene, resulting in motor, behavioral, and cognitive limitations. Boys with dystrophinopathy often exhibit reduced neuropsychological performance in various domains. There remains a lack of research investigating functional and structural brain differences that are crucial for understanding the underlying pathomechanisms to neurobehavioral deficits associated with brain dystrophin loss. Magnetoencephalography (MEG scan) is an imaging technique that identifies brain activity and measures small magnetic fields produced in the brain.
Objective: To explore the functional connectivity using MEG in boys with
dystrophinopathy.
Results: Patients include 5 right handed boys with DMD (Median age M=11.5 years). All patients completed neuropsychological evaluation as part of the study with diagnoses that included ADHD (N=2), Anxiety (N=2), Depression (N=1), and externalizing behavior disorder (N=1).Cognitive screening on the Wechsler Abbreviated Intelligence Scale Second Edition (WASI-II) was overall low average (SS=82.6 +/- 10.85) and generally consistent with average values in youth with DMD. MEG was collected for spontaneous and language protocols. We were able to obtain MEG source analysis for the four participants for spontaneous and language measures. We extracted normalized power spectral density and language brain maps from the four participants in the specific region of interests. Group averaged power of cortical activity extracted from spontaneous data in selected frequency bands shows similar maps reported in the literature. We will perform functional connectivity of the spontaneous data and quantify the receptive language cortical activations to define language dominance based on the laterality index calculated by regions of interests.
Conclusion: Here we report the preliminary findings of MEG in boys with DMD. Further analysis is being conducted. More data will be presented in the conference.