In this study that included 14 DMD patients [young adults (n=7, 18-25 year-old and older adults (n=7, 30-37 year-old) groups] and 16 age-matched normal volunteers, the autgors aimed to determine the distribution of GABAA receptors (GABAA -Rs) in the brain of Duchenne muscular dystrophy (DMD) patients and to ascertain if the distribution pattern correlates with cognitive dysfunction. GABAA -R distribution was assessed using 123 I-IMZ-SPECT. Neuropsychological assessments were performed using 3 different test batteries, WAIS-III, WMS-R, and Wisconsin Card Sorting Test (WCST). All DMD patients showed significant decline in 123 I-IMZ uptake in the prefrontal cortex (P<0.05). While no differences were detected in the WAIS-III and WMS-R, the WCST scores of DMD patients (2.8 ± 1.9) were significantly lower (P<0.01) than those of normal volunteers (5.4 ± 0.7). Both abnormalities were more pronounced in older adult patients. These findings demonstrate that DMD is accompanied by a reduction in the prefrontal cortex distribution of GABAA -Rs.
