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Deep Brain Stimulation of Globus Pallidum Internus in children with dystonia: Effects on motor, cognitive and affective functions

Marotta Rosa

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.  

Biography:

Degree in Medicine and Surgery, University of Naples, Italy; Postgraduate Diploma in Child Neuropsychiatry, University of Naples and in Pediatrics, Faculty of Medicine of Catanzaro, University of Reggio Calabria, Italy; Aggregate Professor of Child Neuropsychiatry. Dr Marotta has her expertise in Clinical-behavioral assessment of individuals with Autism Spectrum Disorder, Evaluation of the evolutionary pathway of subjects affected by neurodevelopmental disorders.

 

7th International Conference on Depression, Anxiety and Stress Management; Barcelona, Spain January 20-21, 2020.

Speaker Publications:

Anxiety and depression levels in prepubertal obese children: a case-control study; M Esposito, B Gallai, M Roccella, R Marotta, F Lavano, SM Lavano; Neuropsychiatric disease and treatment Pervasive developmental disorders in children of hyperandrogenic women with polycystic ovary syndrome: a longitudinal case–control study; S Palomba, R Marotta, A Di Cello, T Russo, A Falbo, F Orio, A Tolino; Clinical endocrinology 77 (6), 898-904 Primary nocturnal enuresis as a risk factor for sleep disorders: an observational questionnaire-based multicenter study; M Esposito, B Gallai, L Parisi, M Roccella, R Marotta, SM Lavano; Neuropsychiatric Disease and Treatment 9, 437 Executive dysfunction in children affected by obstructive sleep apnea syndrome: an observational study; M Esposito, L Antinolfi, B Gallai, L Parisi, M Roccella, R Marotta; Neuropsychiatric disease and treatment 9, 1087 Depressive symptoms and childhood sleep apnea syndrome; M Carotenuto, M Esposito, L Parisi, B Gallai, R Marotta, A Pascotto; Neuropsychiatric disease and treatment 8, 369

Abstract Citation:
Marotta Rosa, Deep Brain Stimulation of Globus Pallidum Internus in children with dystonia: Effects on motor, cognitive and affective functions, Stress 2020, 7th International Conference on Depression, Anxiety and Stress Management; Barcelona, Spain January 20-21, 2020.

(https://stressmanagement.psychiatrycongress.com/abstract/2020/deep-brain-stimulation-of-globus-pallidum-internus-in-children-with-dystonia-effects-on-motor-cognitive-and-affective-functions)