Deki Tsering1, Laura Tochen2, Bennett Lavenstein2,3, Srijaya K Reddy3,4, Yael Granader5, Robert F Keating1,3, Chima O Oluigbo6,7. 1. Division of Neurosurgery, Children's National Health System, 4th Floor, Suite 100, 111 Michigan Avenue NW, Washington, DC, 20010, USA. 2. Division of Neurology, Children's National Health System, Washington, DC, USA. 3. The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. 4. Division of Anesthesiology, Children's National Health System, Washington, DC, USA. 5. Division of Neuropsychology, Children's National Health System, Washington, DC, USA. 6. Division of Neurosurgery, Children's National Health System, 4th Floor, Suite 100, 111 Michigan Avenue NW, Washington, DC, 20010, USA. coluigbo@childrensnational.org. 7. The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. coluigbo@childrensnational.org.
Abstract
PURPOSE: There is a paucity of effective long-term medication treatment for secondary dystonias. In situations where significantly impairing secondary dystonias fail to respond to typical enteral medications and intrathecal (or even intraventricular) baclofen, consideration should be given to the use of deep brain stimulation (DBS). While Level I evidence and long-term follow-up clearly demonstrate the efficacy of DBS for primary dystonia, the evidence for secondary dystonia remains mixed and unclear. In this study, we report our experience with pediatric subjects who have undergone DBS for secondary dystonia. METHODS: We discuss the indications and outcomes of DBS procedures completed at our center. We also present a detailed discussion of the considerations in the management of these patients as well as a literature review. RESULTS: Of the four cases retrospectively examined here, all subjects experienced reductions in the severity of their dystonia (ranging from 0 to 100% on both the Barry-Albright Dystonia (BAD) and Burke-Fahn-Marsden Dystonia Rating Scale-Motor (BFMDRS-M) scales). CONCLUSIONS: Pallidal DBS should be considered among children with functionally debilitating, medication-resistant secondary dystonia. Patients without fixed skeletal deformities who have experienced a short duration of symptoms are most likely to benefit from this intervention.
PURPOSE: There is a paucity of effective long-term medication treatment for secondary dystonias. In situations where significantly impairing secondary dystonias fail to respond to typical enteral medications and intrathecal (or even intraventricular) baclofen, consideration should be given to the use of deep brain stimulation (DBS). While Level I evidence and long-term follow-up clearly demonstrate the efficacy of DBS for primary dystonia, the evidence for secondary dystonia remains mixed and unclear. In this study, we report our experience with pediatric subjects who have undergone DBS for secondary dystonia. METHODS: We discuss the indications and outcomes of DBS procedures completed at our center. We also present a detailed discussion of the considerations in the management of these patients as well as a literature review. RESULTS: Of the four cases retrospectively examined here, all subjects experienced reductions in the severity of their dystonia (ranging from 0 to 100% on both the Barry-Albright Dystonia (BAD) and Burke-Fahn-Marsden Dystonia Rating Scale-Motor (BFMDRS-M) scales). CONCLUSIONS: Pallidal DBS should be considered among children with functionally debilitating, medication-resistant secondary dystonia. Patients without fixed skeletal deformities who have experienced a short duration of symptoms are most likely to benefit from this intervention.
Entities:
Keywords:
Deep brain stimulation; Pediatric neurosurgery; Secondary dystonia
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