Rethinking status dystonicus: A welcome start to a challenging problem

Lumsden, Daniel E.; Allen, Nicholas M.

doi:10.1002/mds.27291

Cited by 14 publications

(12 citation statements)

References 5 publications

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“…anti‐N‐methyl‐D‐aspartate receptor encephalitis in presentations dominated by choreic features). Other open questions include the role of fever, rhabdomyolysis, and metabolic disturbances as supporting criteria to define status dystonicus—as included in the DSAP scoring system . Nevertheless, the main critical issue remains unsolved; namely the duration (or number of episodes) of dystonic exacerbations required to define status dystonicus in an objective and replicable way.…”

Section: Discussionmentioning

confidence: 99%

“…Nevertheless, the main critical issue remains unsolved; namely the duration (or number of episodes) of dystonic exacerbations required to define status dystonicus in an objective and replicable way. The current lack of a robust standardized definition is probable the main barrier to prompt recognition and optimal management of status dystonicus …”

Section: Discussionmentioning

confidence: 99%

“…29 These authors suggested including all potentially lifethreatening hyperkinetic movement disorder emergencies in the broad category of status dyskineticus, regardless of the specific phenomenology or pre-existing condition (if any). However, Lumsden and Allen 30 questioned this definition, arguing that hyperkinetic crises without dystonia should be differentiated from status dystonicus, because of the possibility of a specific underlying condition whose recognition and appropriate management should be emphasized (e.g. anti-N-methyl-D-aspartate receptor encephalitis in presentations dominated by choreic features).…”

Section: Discussionmentioning

confidence: 99%

“…Other open questions include the role of fever, rhabdomyolysis, and metabolic disturbances as supporting criteria to define status dystonicus-as included in the DSAP scoring system. 29,30 Nevertheless, the main critical issue remains unsolved; namely the duration (or number of episodes) of dystonic exacerbations required to define status dystonicus in an objective and replicable way. The current lack of a robust standardized definition is probable the main barrier to prompt recognition and optimal management of status dystonicus.…”

Section: Discussionmentioning

confidence: 99%

“…The current lack of a robust standardized definition is probable the main barrier to prompt recognition and optimal management of status dystonicus. 30 Further limitations mainly exist in the retrospective design and the small sample size. First, during the study period no routine protocol for dystonia exacerbation management was applied at our institution, making assessment and management of our cohort highly heterogeneous.…”

Section: Discussionmentioning

confidence: 99%

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Prestatus and status dystonicus in children and adolescents

Garone

Graziola

Nicita

et al. 2019

Develop Med Child Neuro

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Prestatus and status dystonicus in children and adolescents

Garone

Graziola

Nicita

et al. 2019

Develop Med Child Neuro

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Deep Brain Stimulation for Refractory Status Dystonicus in Children: Multicenter Case Series and Systematic Review

Vogt,

Yan,

Santyr

et al. 2023

Annals of Neurology

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ObjectiveWe sought to better understand the workflow, outcomes, and complications of deep brain stimulation (DBS) for pediatric status dystonicus (SD). We present a systematic review, alongside a multi‐center case series of pediatric patients with SD treated with DBS.MethodsWe collected individual data regarding treatment, stimulation parameters, and dystonia severity for a multi‐center case series (n=8) and all previously published cases (n=77). Data for case series was used to create probabilistic voxel‐wise maps of stimulated tissue associated with dystonia improvement.ResultsIn our institutional series, DBS was implanted a mean of 25 days post‐SD onset. Programming began a mean of 1.6 days after surgery. All eight patients in our case series and 73/74 reported patients in the systematic review had resolution of their SD with DBS, most within 2‐4 weeks of surgery. Mean follow‐up for patients in the case series was 16 months. DBS target for all patients in the case series and 68/77 in our systematic review was the globus pallidus pars interna (GPi). In our case series, stimulation of the posterior‐ventrolateral GPi was associated with improved dystonia. Mean dystonia improvement was 32% and 51% in our institutional series and systematic review, respectively. Mortality was 4% in the review, which is lower than reported for treatment with pharmacotherapy alone (10‐12.5%).InterpretationDBS is a feasible intervention with potential to reverse refractory pediatric SD and improve survival. More work is needed to increase awareness of DBS in this setting, so that it can be implemented in a timely manner.This article is protected by copyright. All rights reserved.

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Status Dystonicus

Meijer

Fasano

2021

Movement Disorder Emergencies

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Rethinking status dystonicus: A welcome start to a challenging problem

Cited by 14 publications

References 5 publications

Prestatus and status dystonicus in children and adolescents

Prestatus and status dystonicus in children and adolescents

Deep Brain Stimulation for Refractory Status Dystonicus in Children: Multicenter Case Series and Systematic Review

Status Dystonicus

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