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This study aims to assess the family functioning and health-related quality of life (HRQOL) in Chinese boys with Duchenne muscular dystrophy (DMD) and their parents using Pediatric Quality-of-Life Family Impact Module ( PedsQL FIM) and Pediatric Quality-of-Life Inventory (PedsQL) 4.0. Findings from 15 families with DMD were compared with 15 unaffected families. The HRQOL, as measured by the mean PedsQL 4.0 Generic Core Scale scores for the boys with DMD were significantly lower than those of age-matched healthy boys, for overall ( p < 0.05, parent-report; p <0.001, self-report), physical ( p < 0.001, parent-report and self-report), and social ( p < 0.05, parent-report) functioning, but the emotional functioning is not affected. The parent-child concordance of our affected DMD families was generally in the moderate-to-good agreement range (intraclass correlation coefficients from 0.51 to 0.73), except for emotional (0.28) and social (0.31) functioning. The PedsQL FIM total score showed an inverse relationship with the affected child's age (correlation coefficient: -0.55; p < 0.01) and the disease stage (correlation coefficient: -0.63; p < 0.01) confirming that parental HRQOL and overall family functioning worsened as the child increased in age with advancing disease stage.

This study aimed to evaluate P300 as an electrophysiological marker of cognitive function in patients with systemic lupus erythematosus (SLE) who had previous neuropsychiatric (NPSLE) involvement and were diagnosed to have cognitive impairment by standard neuropsychological tests. Event-related potentials (ERPs) were assessed by the auditory and visual oddball paradigms. Amplitude and latency of P300 at the frontal (Fz), central (Cz), and parietal (Pz) regions were determined and compared with controls. P300 detection was performed in NPSLE patients with pre-diagnosed cognitive impairment (n = 9), matched SLE patients without previous NPSLE (non-NPSLE) (n = 9), and healthy controls (n = 15). Auditory oddball task did not show any P300 abnormality between groups. Visual oddball task revealed reduced amplitude of P300 over Fz ( P = .002) and Cz ( P = .009) electrodes in NPSLE patients compared with healthy controls and among those who had predominant memory deficit ( P = .01 at Fz). Abnormal P300 was also observed in non-NPSLE patients at Fz and Cz. Using visual oddball paradigm, abnormal P300 was found in NPSLE patients over frontal and parietal regions compared with normal controls but was not discriminative from possible subclinical disease in non-NPSLE patients. In conclusion, visual oddball paradigm was a more sensitive electrophysiological marker than auditory oddball paradigm for cognitive impairment in NPSLE patients.