Objective: Retrospective analysis of an 8-years’ experience in diagnosis, management and follow-up of children experiencing Status dystonicus (SD) and pre-SD.

Background: SD is considered the most severe form of a continuum spectrum of worsening dystonia, and pre-SD represents the immediately preceding step in acute deterioration. SD and pre-SD occur more frequently in children with a long duration of dystonia, but few case series have been reported[1-3].

Method: Retrospective analysis of medical records of patients hospitalized for acute exacerbation of dystonia (January 2010-August 2018). Clinical, demographic and therapeutic features were analysed according with disease severity, scored on a 3 points rating scale. For patients with at least 6 months of follow-up, risk of subsequent relapse was estimated by means of Kaplan-Meier curves.

Results: 34 patients (8 females) experiencing 63 episodes of pre-SD and SD were identified. Mean age at presentation was 9 years 11 months (range 1-20 years). Dystonic symptoms onset dated back to infancy in the large majority of cases. 14 patients experienced ≥2 episodes (mean 1.82 episode/patient, 2 episode/year of follow-up). In 18 cases ICU admission was needed. Infection were the most commonly trigger (47.6%). In about three quarters of the cases a baseline antidystonic therapy was already ongoing at the time of acute exacerbation. Benzodiazepines, both for oral and IV administration, were the most commonly used drugs. Pallidotomy was performed in 6 cases during SD, and 2 additional patients underwent elective pallidotomy after having obtained SD remission by medical therapy. 6 of them developed subsequent relapses.27 patients were eligible for Kaplan-Meier curve analysis. The probability of survival free from SD relapses was 77.7% after 4 months, 73.5% after 13 months and 61.4% after 27 months.

Conclusion: Dystonia exacerbations are potentially life-threating emergencies, mainly affecting individuals with early-onset dystonia. Patients experiencing pre-SD and SD have a considerable risk of developing further episodes. Nevertheless, no obvious factor for relapse risk stratification exist. Pallidotomy is a feasible option in medical refractory SD for patients with limited deep brain stimulation applicability, but the risk of recurrence is elevated. Preliminary results of our study have been already presented at the 6th Symposium on Paediatric Movement Disorders (Barcelona, 2019).

References: [1] Fasano A, Ricciardi L, Bentivoglio AR, et al. Status dystonicus: predictors of outcome and progression patterns of underlying disease. Mov Disord. 2012;27(6):783-788. doi:10.1002/mds.24981 [2] Lumsden DE, King MD, Allen NM. Status dystonicus in childhood. Curr Opin Pediatr. 2017;29(6):674-682. doi:10.1097/MOP.0000000000000556 [3] Nerrant E, Gonzalez V, Milesi C, et al. Deep brain stimulation treated dystonia-trajectory via status dystonicus. Mov Disord. 2018;33(7):1168-1173. doi:10.1002/mds.27357

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MDS Abstracts - https://www.mdsabstracts.org/abstract/pre-status-and-status-dystonicus-in-children-and-adolescents/