INTELLECTUAL DEVELOPMENT OF PATIENTS WITH PROGRESSIVE DUSCHENNE AND BECKER MUSCULAR DYSTROPHIES

Аннотация

Тo assess the intellectual level of 166 patients with PMDD and 15 patients with PMDB, experimental psychological testing was performed using the Raven matrix (intelligence test). Intellectual insufficiency in Duchenne muscular dystrophy, found in literary data in 30-60%, in our sample was 43% (71 patients out of 166 examined). The main cause of the intellectual defect in Duchenne muscular dystrophy is the deficiency of cerebral isoforms of dystrophin - apodystrophins, the synthesis of which is disturbed in the case of distal deletions of the dystrophin gene (deletion of exons 40-53).