Introduction: Deep Brain Stimulation (DBS) of the Globus Pallidus Interna has been shown to be extremely effective in primary generalized dystonia. There is much less evidence for the use of DBS in patients with secondary dystonia. We present a series of 14 patients with secondary dystonia who underwent pallidal DBS at our institution.
Methods: A retrospective review of patients with secondary dystonia who received treatment with DBS between February 2011 – December 2014 was performed. Pre-operative and post-operative videos were scored using the Barry Albright Dystonia (BAD) score. In addition, a Caregiver Assessment Score for Dystonia(CAS-D) was completed using the following scale: -1(worse), 0(no benefit), 1(minimal benefit), 2(good benefit), 3(excellent benefit).
Results: BAD scores improved by 4% (p=0.07) with minimal improvement in patients with kernicterus (n=3, 0% improvement) and the most improvement in dystonia secondary to drug overdose (n=1, 22% improvement). However, even with 0% BAD improvement, patients with kernicterus had life-impacting changes that were not reflected by BAD scores: patient A had less opisthotonus with a significant decrease in respiratory complications, patient B was able to dress herself, and patient C was able to independently drive his wheelchair after DBS treatment. In addition, even in patients with minimal change on BAD scoring, caregivers reported decreased contractures and spasms leading to improved comfort levels. The mean CAS-D score was 1.4 with 2, 4, 6, and 2 caregivers reporting excellent, good, minimal and no benefit respectively. In addition, there were no complications such as infections or hematoma in our series.
Conclusions: DBS does provide benefit to a segment of the secondary dystonia population. Current dystonia scoring does not adequately reflect patient improvement after DBS. Larger studies for secondary dystonia focusing on age, etiology, and patterns of dystonia are needed to learn which patients will respond best to treatment.
Patient Care: Improved indications for DBS in children and young adults
Learning Objectives: Indications for DBS in children