Objective: To develop consensus recommendations for the assessment, triaging and management of pre-status dystonicus, status dystonicus, and refractory status dystonicus in children.

Background: Status dystonicus (SD) is the most severe form of dystonia and a medical emergency with significant morbidity. SD is especially common in the pediatric age group and despite previous publications providing general recommendations, there is still a need for comprehensive and systematic guidelines.

Method: To address this unmet need, interdisciplinary working groups from two North American centers undertook a literature review, evaluated institutional experiences, and developed two clinical pathways. These pathways underwent a multistep consensus process, refining them based on multidisciplinary input provided by relevant teams, stakeholders and patient safety and quality improvement initiatives. Following implementation of the pathways, a number of metrics were collected to measure the pathway’s impact on the care of children admitted with SD.

Results: We present guidelines that outline a stepwise approach to interdisciplinary treatment and monitoring of SD in children. These clinical pathways aim to: 1) facilitate timely recognition/triaging of worsening dystonia, 2) standardize supportive and dystonia-directed therapies, 3) provide structure for interdisciplinary cooperation, 4) integrate advances in genomics and neuromodulation, 5) enable multi-center quality improvement and research, and 6) improve outcomes. Following implementation, we have been tracking metrics to measure the pathway’s impact including utilization in different care setting, length of hospital stay, admissions to the ICU, need for intubation and sedative infusions, and pre- versus post-SD dystonia severity.

Conclusion: Our pathways present consensus recommendations for managing the spectrum from pre-SD to refractory SD in children, addressing SD as a neurological emergency. Our preliminary work suggests increased familiarity with dystonia among care teams. We hypothesize that longitudinal analysis will establish metrics of quality improvement, providing a foundation for further refinement of these guidelines.

Acute Dystonia Pathway

Refractory Status Dystonicus Pathway

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-impact-of-standardized-recommendations-on-the-management-of-initial-and-refractory-pediatric-status-dystonicus/