Visual/verbal cross-modal memory was assessed by means of the Memory for Names Subtest of the Test di Memoria e Apprendimento battery (an Italian adaptation of the Test of Memory and Learning) [35], requiring subjects to learn and recall the names of eight children whose faces are depicted

in line drawings. Verbal learning, supraspan memory, and resistance to interference were assessed via the List Learning Subtest of the Test of Memory and Learning [35]. This test assesses the TAM Receptor inhibitor immediate recall of word lists over repeated trials with or without interference, as well as a delayed recall after 30 minutes. Visual memory was assessed with the Abstract Visual Memory Subtest from the Test of Memory and Learning [35], requiring the immediate recognition of abstract, nonverbalizable pictures. Selleckchem Gefitinib All scores are expressed as z-scores. The two groups of children with Duchenne muscular dystrophy with distal and proximal mutations were compared in

terms of various cognitive and neuropsychologic measures. Subsequent analyses were performed to better define: (1) the degree of impairment of the two subgroups compared with control patients, and (2) the relationship of the deficits highlighted in either subgroup with other theoretically relevant cognitive and neuropsychologic functions. SPSS software (SPSS, Inc, Chicago, IL) was used for all analyses. Our study showed that in Italian-speaking children with Duchenne muscular dystrophy, the intelligence quotient is approximately 1 S.D. below the Inositol monophosphatase 1 population average, with an overall mean full-scale intelligence quotient of 86.43 ± 13.7, and a discrepancy between verbal intelligence quotient (86.26 ± 14.9) and performance intelligence quotient (89.98 ± 14.8). The control group of patients with spinal muscular atrophy or osteogenesis imperfecta (severely motor impaired) did not manifest any cognitive deficits, with a full-scale intelligence quotient of 107.7 ± 10.45, a verbal intelligence quotient of 108 ± 9.34, and a performance intelligence

quotient of 105.6 ± 16. Separate analyses, taking into account genetic alterations in the dystrophin gene (Duchenne muscular dystrophy distal and Duchenne muscular dystrophy proximal), indicated that the verbal intelligence quotients of both groups were significantly lower than those of the control children, whereas only distally mutated children with Duchenne muscular dystrophy demonstrated significantly lower performance intelligence quotients (Table 1). In the Duchenne muscular dystrophy distal group, 24 of 25 patients carried mutations predicted to affect all dystrophin products, including Dp140 but not Dp71. Only one patient carried a mutation also affecting Dp71. That patient did not exhibit deficits in any neurocognitive function. Moreover, his full-scale intelligence quotient was above 100.