ICNC2018 Abstracts & Symposia Proposals, ICNC 2014

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Pediatric Dystonia: DBS and Diversity in Diencephalic Dysfunction
Aaron L Cardon, Amber J Stocco

Building: Bourbon Cataratas Convention Centre, Foz do Iguaçu
Room: Cataratas I
Date: 2014-05-05 02:30 PM – 02:45 PM
Last modified: 2014-02-09

Abstract


Deep-brain stimulation (DBS) use in pediatric centers began in 2007, ten years after use in adults proved efficacious and safe in treating movement disorders.  Specific challenges in using DBS to treat pediatric dystonia include careful patient selection, cooperation in intraoperative clinical assessment and pediatric adjustments to microelectrode recording. We present our experience over four years in selection, treatment success, and continuing medical management of a series of patients offered DBS for dystonia of various etiologies.  After rigorous review at the Texas Children’s Hospital Spasticity and Dystonia Multidisciplinary Review Committee and Baylor-wide DBS Consensus conferences, approximately 30 children have been offered DBS at our center. 12 patients with medically-intractable primary or secondary dystonia, between 6-20 years old, have undergone the procedure.  Their condition results from broad underlying pathologies: 4 have primary genetic etiologies (2 pantothenate kinase deficiency, 1 DYT1, and 1 macrodeletion including 3q29), 5 have cerebral palsy (3 term HIE, 1 PVL from extreme prematurity, and 1 intrauterine stroke), and 1 patient each had stroke, necrotizing ADEM, or persistent tardive dyskinesia after risperidone. Treatment goals individualized to presurgical function and symptoms were used to evaluate outcomes. Most have improved target symptoms, reduced medications, and increased mobility and independence. Children with HIE and the macrodeletion have less favorable results. 1 patient had ICH, 2 developed infection requiring implant removal, and 3 others underwent revision for improved symptom control.  Emerging from diverse pathologies in highly-conserved, compact, surgically-amenable targets, dystonias highlight the usefulness of DBS in treating static and progressive neurological diseases.


Keywords


Dystonia; deep brain stimulation; cerebral palsy; genetic dystonia

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