Objective: To present a case of deep brain stimulation (DBS)-ameliorated myoclonus-dystonia syndrome secondary to acute disseminated encephalomyelitis (ADEM) and characterize fibers and circuits affected by stimulation.

Background: DBS is an established and effective therapy for movement disorders. Here we present a case of secondary myoclonus-dystonia syndrome following ADEM in childhood, which was alleviated by DBS.

Method: A 19-year-old man presented with several years of progressive dystonia and myoclonic jerks in his right arm, posture tremor, akathisia, and impaired concentration. Brain-MRI revealed lesions in the pons, cerebellum, thalamus, and periventricular medullary. The patient received unilateral lead placement in the left globus pallidus internus (GPi) after undergoing a multidisciplinary evaluation and not responding to anticonvulsant and anticholinergic medication.

Results: Motor assessments utilizing the Burk-Fahn-Marsden Dystonia Rating Scale (BFMRS) and the Unified Myoclonus Rating Scale (UMRS) indicated that the patient experienced a greater improvement in dystonia compared to myoclonus following adjustments to the DBS stimulation parameters based on the visualization of electrode position and volume of tissue activated (VTA) twelve months after surgery. A patient-specific connectome analysis using the VTA as a region of interest revealed fiber tracts connecting to the cerebello-thalamo-cortical network and the superior frontal gyrus in addition to basal ganglia circuits as particularly effective.

Conclusion: This case study suggests that GPi DBS may be considered in secondary myoclonus-dystonia syndromes, and that personalized structural considerations are necessary due to individual symptoms and clinical characteristics. Patient-specific connectome analysis can provide insights into the structures involved in a beneficial response.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/unilateral-gpi-dbs-in-secondary-myoclonus-dystonia-syndrome-after-acute-disseminated-encephalomyelitis-a-case-report/