Objective: To identify distinct pallidal local field potential (LFP) signatures characterizing status dystonicus (SD) from non-SD dystonia.

Background: SD is a poorly understood neurological emergency requiring urgent interventions, including globus pallidus interna (GPi) deep brain stimulation (DBS). Sensing-capable DBS electrodes provide an opportunity to study SD pathophysiology. Despite increasing use of pallidal DBS, oscillatory & non-oscillatory electrophysiological signatures of SD remain unknown. We sought to identify intracranial neural biomarkers of SD from a rare cohort of children, critical since children at risk often have chronic baseline dystonia difficult to differentiate from SD.

Method: Ten patients (age: 7.8+/-3.6) with SD (Hospital for Sick Children, Canada) were implanted with Medtronic Percept™ GPi DBS [figure1](A). LFPs were recorded longitudinally during SD, recovery and relapse (range: 11-1155 days) and Power Spectral Densities (PSDs) calculated (Welch-method). Analysis of oscillatory and aperiodic activity was performed [figure2](A) (fitting-oscillations/one-over-f). Band-limited power (Theta: 3-7Hz; Alpha: 7-12.5Hz, Beta: 12.5-30Hz) was calculated. Bursting activity within +/-3 Hz around the beta peak frequency was computed [figure3](A). Generalized linear mixed effects models assessed relations between LFP metrics and clinical scales, including Burke‐Fahn‐Marsden Dystonia Rating Scale (BFMDRS) and Pediatric Quality of Life Score (PedsQL). Circadian LFP dynamics were assessed in one participant with narrowband beta-band recordings over months.

Results: During SD, periodic activity in the beta-band increased across all patients and recordings [figure1](B), with increased periodic peak power [figure2](B) (p=0.007) and beta-band burst amplitude [figure3](B&C) (p<0.001). Relapsed SD was characterized by return of excessive beta signatures [figure1](C). Beta-specific LFP power alone was significantly associated with worse quality-of-life scores [figure4] (PedsQL, p=0.03, R²=0.695). Circadian beta-band periodicity was present [figure5](A), with significantly increased power across daytime (p<0.001) and nighttime (p<0.001) during SD [figure5](B).

Conclusion: SD is a distinct state with important implications for dystonia pathophysiology, tracking dystonia-states from intracranial activity and potential adaptive DBS treatments.

figure1

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figure5

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