The Language Competence Evaluation And Brain Functional Magnetic Resonance Imaging Study For Duchenne Muscular Dystrophy Children

Background Cognitive impairment is one of the clinical features of Duchenne Muscular Dystrophy(DMD)patients,especially deficit in verbal and intellectual.A large number of studies in abroad shows that DMD patients have language competence impairment,but there has no related research in our country.The resting-state functional MRI(rs-f MRI)is used to investigate the spontaneous activity of the brain at resting-state.It is very suitable to be applied to the DMD patients who have cognitive impairment.Diffusion Tensor Imaging(DTI)is a currently the only effective and non-invasive examination method to observe and track the central nervous system fibers.It is unclear whether f MRI and DTI technology could found the cognitive and verbal impairment evidence in DMD brain.Objective Intelligent scales and verbal testing are used to assess the cognitive and verbal skills in DMD patients,and using f MRI and DTI to examine the features of brain structure and brain function in resting state in subjects with DMD,and explore new clue to neurobiological basis of DMD,in order to provide evidence of verbal impairment.Methods All patients who were diagnosed as DMD were recruited in the DMD joint clinic in the General Hospital of Chinese Armed Police Force.All control group members were recruited from social family and their gender,age and years of education were matched with DMD boys.Respectively,two groups were tested with Wechsler intelligence assessments(including total intelligence,Verbal intelligence,Performance intelligence)and language skills test(including picture-naming,syntactical tests,Syllable deletion,vocabulary definitions).Meanwhile.All participants had underwent the line axial T2 WI,axial T1 WI,coronal FLAIR,and DTI scanning with a 3T MR imager.The structure,functional and DTI data in the whole brain were obtained.Then we analyzed the microstructure changes in the whole brain gray matter and white matter,and detected the brain parts and white matter fibers which with significant differences between two groups under resting state.The correlation between these changes and cognitive scores of DMD patients were further studied.Results(1)Cognitive and language: There are 63 DMD cases,14 cases of normal children in the control group.There has great significant difference between two groups in IQ scores.The Wechsler Total IQ,Verbal IQ,and Performance IQ in DMD group were significantly lower than the control group(p <0.001).Language skills test were significantly lower than normal reference values(p <0.001),especially in the Vocabulary definition.(2)rs-f MRI: There are 48 DMD cases,12 cases of normal children in the control group.AM value in the thalamus and temporal pole in DMD group was significantly lower than that in the control group(p <0.001,FDR = 0.05 corrected);Re Ho value in the thalamus in DMD group was significantly lower than that in the control group,while significantly higher in the left temporal lobe than that in control group(p <0.01,FDR = 0.05 correction).(3)DTI: There are only 47 DMD cases and 9 cases of normal children in the control group.FA value in the Posterior limb of internal capsule R in DMD group was significantly lower than that in the control group(p<0.01,FDR = 0.05 correction).It was significantly higher than that in the control group in inferior cerebellar peduncle R,Superior cerebellar peduncle L,Sagittal stratum R/L,External capsule R/L,Cingulum(hippocampus)L,Superior longitudinal fasciculus L(P<0.05).Conclusion(1)Some Chinese DMD patients have cognitive and language function impairment,which is mainly manifested on expressive language impairment.(2)There exist abnormalities in neural spontaneous activity on the thalamus and temporal pole in DMD patients,which may be one of the neurobiological mechanisms that cause abnormality in neurobehavioral abnormal in DMD patients.(3)It is found in China that DMD patients had microstructural changes in the brain,especially in Posterior limb of internal capsule.This may be a compensatory mechanism of brain for motion function impairment.