Objective: Assess the utility of deep brain stimulation (DBS) in treatment of chorea in pediatric patients.

Background: While DBS has become common in treatment of dystonia in Parkinson’s disease and certain genetic conditions, it is not widely utilized in treatment of chorea. The majority of the literature on its utilization centers on treatment of adults with chorea-acanthocytosis or Huntington’s disease, with efficacy in these conditions mixed(1)(2). Literature in pediatrics is sparse and predominantly case series related to treatment of choreiform movements in children with cerebral palsy(3) and ADCY5(4) . Here we present two subjects with relatively pure chorea of different etiologies with beneficial response to DBS.

Method: Retrospective video and chart analysis of two subjects prior to DBS and 6 months postoperatively from placement of electrodes bilaterally in globus pallidus internus (GPi) and ventralis intermediate nuclei (VIM) of thalamus.  Subjects were identified as having chorea by a pediatric movement disorders specialist based on presence of ongoing random-appearing sequence of multiple discrete repeated involuntary nonrhythmic movements(5).

Results: Subject 1 is a male with post-pump chorea secondary to Williams Syndrome. He was implanted at age 5 due to severe chorea placing himself at risk for harm and preventing weight gain. He was noted to have 33% decrease in chorea as measured by the Abnormal Involuntary Movement Scale (AIMS) 6 months postoperatively, and a 5 point decrease in the motor component of Unified Huntington’s Disease Ratings Scale (UHDRS.) Subject 2 is a male with chorea secondary to KMT2B mutation implanted at age 10 due to severe chorea causing multiple hospitalizations and extremity fracture. He had a 65% decrease in chorea based on the AIMS six months postoperatively, and a 24 point decrease in the motor component of the UHDRS. Both subjects have sustained this improvement greater than 1 year postoperatively.

Conclusion: In treatment-resistant chorea deep brain stimulation of the GPi and VIM should be considered as may provide substantial benefit for patients. This is noted in two patients with chorea of different underlying etiologies. Further evaluation is indicated to determine if additional targets may be beneficial, particularly in other etiologies based on pathophysiology.

References: 1. Edwards TC, Zrinzo L, Limousin P, Foltynie T. Deep brain stimulation in the treatment of chorea. Movement Disorders [Internet]. 2012 Mar 1 [cited 2023 Mar 12];27(3):357–63. Available from: https://onlinelibrary.wiley.com/doi/full/10.1002/mds.23967
2. Shin H, Ki CS, Cho AR, Lee J Il, Ahn JY, Lee JH, et al. Globus Pallidus Interna Deep Brain Stimulation Improves Chorea and Functional Status in a Patient with Chorea-Acanthocytosis. Stereotact Funct Neurosurg [Internet]. 2012 Aug [cited 2023 Mar 12];90(4):273–7. Available from: https://www.karger.com/Article/FullText/338216
3. Thompson TP, Kondziolka D, Albright AL. Thalamic stimulation for choreiform movement disorders in children: Report of two cases. J Neurosurg [Internet]. 2000 Apr 1 [cited 2023 Mar 12];92(4):718–21. Available from: https://thejns.org/view/journals/j-neurosurg/92/4/article-p718.xml
4. Dy ME, Chang FCF, De Jesus S, Anselm I, Mahant N, Zeilman P, et al. Treatment of ADCY5-Associated Dystonia, Chorea, and Hyperkinetic Disorders With Deep Brain Stimulation: A Multicenter Case Series.
5. Sanger TD, Chen D, Fehlings DL, Hallett M, Lang AE, Mink JW, et al. DEFINITION AND CLASSIFICATION OF HYPERKINETIC MOVEMENTS IN CHILDHOOD.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/treatment-of-chorea-in-pediatric-subjects-with-pallidal-and-thalamic-deep-brain-stimulation/