Objective: We present four cases of pediatric chorea managed with deep brain stimulation (DBS) and review previously reported cases in the literature. We review clinical course and response to treatments as well as stimulation parameters. Video segments are included.

Background: Chorea is a movement disorder characterized by involuntary, non-rhythmic, non-patterned movements that flow from one area to another. Etiology includes structural insults, autoimmune diseases, neurodegenerative diseases, metabolic and genetic conditions. Rarely, disease specific treatments are available, and chorea is often treated symptomatically with medications. In some patients, medications are ineffective and surgical therapy is considered.

Method: literature survey of deep brain stimulation and chorea was performed on PubMed. Additionally, we describe four cases. Three had inherited monogenetic conditions with static encephalopathy (2 cases GNO1, 1 case FOXG1) and one with acquired/structural cause of chorea (pilocytic astrocytoma). The GNO1 patients were both implanted with bilateral GPi-DBS emergently in the setting of life threatening, refractory chorea resulting in rhabdomolysis. patient with FOXG1 had chorea that was pharmacoresistant and impeding cares with subsequent implantation of bilateral GPi-DBS. The last patient presented with hemichorea secondary to pilocytic astrocytoma following partial surgical resection and poor tolerance to oral medications, right GPi-DBS and right posterior subthalamic area (PS) DBS was implanted.

Results: The patients with GNAO1 had significant improvement in chorea; most notably, one patient had no further recurrence of life-threatening chorea with febrile illness while the other had significant reduction in the frequency of these (pre-implantation had 3-4 ICU admissions per year, post-implantation had two admissions over the span of four years). The patient with FOXG1 had mild improvement in chorea. The patient with structural cause of chorea had subjective improvement with GPi stimulation and no change with PSA stimulation. Adverse effects included tract hemorrhage related to implantation in one patient.

Conclusion: In cases of chorea resistant to pharmacologic therapy, DBS is often considered. GPi-DBS may be helpful for inherited monogenetic causes of chorea with static encephalopathy while acquired and neurodegenerative conditions have a less robust response.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-in-chorea-four-cases-and-literature-review/