Prestatus and status dystonicus in children and adolescents

Abstract

Aim To critically analyse the management of status dystonicus and prestatus dystonicus in children and adolescents, in order to examine clinical features, acute management, and risk of relapse in a paediatric cohort. Method Clinical, demographic, and therapeutic features were analysed according to disease severity. Risk of subsequent relapse was estimated through Kaplan–Meier curves. Results Thirty‐four patients (eight females, 26 males) experiencing 63 episodes of acute dystonia exacerbations at a tertiary referral Italian hospital were identified. Mean age at status dystonicus presentation was 9 years 11 months (11y at inclusion in the study). Onset of dystonia dated back to infancy in most cases. Fourteen patients experienced two or more episodes. Infections were the most common trigger (48%). Benzodiazepines were the most commonly used drugs for acute management. Stereotactic pallidotomy was performed in six cases during status dystonicus, and in two additional patients it was electively performed after medical management. The probability of survival free from status dystonicus relapses was 78% after 4 months and 61% after 27 months. Interpretation Dystonia exacerbations are potentially life‐threating emergencies, with a considerable risk of relapse. Nevertheless, no obvious factors for relapse risk stratification exist. Pallidotomy is a feasible option in medical refractory status dystonicus for patients with limited deep brain stimulation applicability, but the risk of recurrence is elevated. What this paper adds Acute exacerbations may affect up to 10% of children with dystonia. Infections are the most common precipitant factor. In about 30% of the cases, intensive care unit admission is needed. Subsequent relapses are common, reaching 25% risk at 1 year. Pallidotomy can be considered in medical‐refractory cases with no deep brain stimulation applicability.

Bilateral Pallidotomy for Dystonia: A Systematic Review

“…However, due to scarcity of literature, non‐English papers were also assessed. Finally, 33 papers met our inclusion criteria and were included in this review 15,17‐48 . The reasons to exclude the other 133 articles were the following: congress abstracts (n = 37), described no patients (n = 25), full text not available (n = 18), unilateral pallidotomy (n = 15), Parkinson's disease (n = 12), no (motor) outcomes (n = 9), patients described before (n = 7), thalamotomy or other lesions (n = 6), no dystonia patients (n = 2), and DBS only (n = 2) ( FIGURE S1).…”

“…In 19 of 100 patients, a relapse of dystonic symptoms was reported (Table 1). One study reported relapsing SD 45 . Six patients were treated with DBS after bilateral pallidotomy, because their dystonic symptoms reappeared after temporary benefit 24,38,49 .…”

“…One study reported relapsing SD. 45 Six patients were treated with DBS after bilateral pallidotomy, because their dystonic symptoms reappeared after temporary benefit. 24,38,49 The relapse time of dystonic symptoms after pallidotomy varied between 3 weeks and 4.5 years.…”

“…Remarkably, in a single study by Garone and colleagues after initial benefit, relapse of symptoms was reported in 7 patients. 45 Unfortunately, the (possible) explanations for these relapses are not mentioned, nor the duration of the relapse nor the type of dystonia. In combination with the fact that this is the only study that reports the recurrence of SD after bilateral pallidotomy, conclusions cannot be drawn on the risk of relapse after SD.…”

The spectrum of dystonia severity before status dystonicus

“…The reported mortality of status dystonicus has remained concerningly high at 10%, with current guidelines for management entirely based on expert opinion. Garone et al report the management and clinical course of 34 children experiencing 64 episodes of acute dystonia that required hospital admission. This report is welcome, not only for contributing to the sparse literature on status dystonicus, but also for the explicit demonstration that not all presentations with worsening dystonia are at the point at which they are actively life threatening.…”