Marotta Rosa
Pugliese Hospital, Italy
Title: Deep Brain Stimulation of Globus Pallidum Internus in children with dystonia: Effects on motor, cognitive and affective functions
Biography
Biography: Marotta Rosa
Abstract
Pediatric Dystonia is a syndrome of sustained muscle contractions producing abnormal postures, twisting, and repetitive movements. A variety of forms have been described, most of which are refractory to medical therapy alone. Deep Brain Stimulation (DBS) has been used to treat several of these conditions but the success varies according to the type of dystonia. Were enrolled 9 pediatric patients with generalized dystonia, 4 males and 5 females; 6 patients had a primary dystonia and 3 a secondary dystonia. The mean age at surgery was 16 years, ranging from 14 to 17 years. Among the patients suffering from primary dystonia, 4 were tested positive and 2 negative for the mutation of DTYI gene located on chromosome 9q34. No patients suffering from secondary dystonia were tested positive to the mutation of DTYI gene. The patients were subjected to bilateral Globus Pallidus Internus (GPi) DBS. While patients with primary dystonia appeared to have superior results compared to those with secondary forms of the disease, the latter patients, in some cases, may still be candidates for DBS The DBS of GPi in dystonias in developmental age had effects not only on motor function, but also on cognitive and affective ones. In most cases these functions remained stable or better, rarely worsed. The improvement of the motor component in itself positively affects the remaining functions and the consequent reduction in drug therapy improves the performances in the course of cognitive evaluation. While putting together the cost of complementary therapeutical approaches in childhood dystonia, DBS can represent a reasonable approach when candidates are well selected and requirements related to selection criteria fullfilled.
Treating dystonia in children with DBS requires a multidisciplinary team to select candidates, identify reasonable target symptoms, perform the procedure, cope with complications related to therapy and devices, optimize DBS and medical therapy administration, support the patients and families over the changing life. Although this is an effective and reversible procedure, we must always reflect on the possible effects on the developmental trajectory of each subject in the development phase.