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Pediatric Status Dystonicus: A Single-Center 10-Year Retrospective Review
Dakota J S J Peacock1, Keshvi Vithlani2,3, Grace A Clarke3,4
1Division of Neurology, Department of Pediatrics, BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Insights
Status dystonicus is a severe, life-threatening movement disorder emergency in children. This study highlights common triggers like infection and pain, emphasizing the need for standardized treatment guidelines to improve outcomes.
Area of Science:
- Pediatric Neurology
- Intensive Care Medicine
- Movement Disorders
Background:
- Status dystonicus is a critical medical emergency characterized by severe, generalized dystonia.
- The condition's heterogeneous nature, including its causes and outcomes, complicates standardized management.
- There is a need to better understand the characteristics and care of pediatric patients experiencing status dystonicus.
Purpose of the Study:
- To characterize the demographics of pediatric patients with status dystonicus admitted to a pediatric intensive care unit (PICU).
- To analyze management patterns and clinical outcomes for children experiencing status dystonicus.
- To identify common triggers and treatment approaches for status dystonicus in a tertiary care setting.
Main Methods:
- Retrospective review of clinical records at a tertiary children's hospital PICU from 2014 to 2024.
- Inclusion criteria: patients aged >30 days with dystonia severity grade 3-5, or worsening dystonia.
- Data collection included demographics, etiology, triggers, medications, PICU stay duration, and mortality.
Main Results:
- Forty-one admissions from 19 unique pediatric patients were analyzed; mean age was 7.6 years, with 53% females.
- Genetic etiology was common (42%), and infection (56%) or pain (15%) frequently triggered admissions.
- Patients received multiple anti-dystonia medications; median PICU stay was 7 days, with a 4.9% mortality rate.
Conclusions:
- Status dystonicus is a life-threatening pediatric emergency often precipitated by infection or pain.
- The study underscores significant morbidity and mortality associated with status dystonicus.
- Multidisciplinary teams should consider developing standardized treatment guidelines for managing these complex pediatric cases.
Background:
Status dystonicus is a life-threatening movement disorder emergency characterized by frequent or prolonged severe episodes of generalized dystonia. The phenomenology, etiology, and outcome is heterogenous and poorly characterized, making implementation of a standardized management approach challenging.
Objectives:
We characterized demographics of children with status dystonicus in a tertiary children's hospital pediatric intensive care unit (PICU), management patterns, and outcomes.
Methods:
Clinical records at our PICU were searched via ICD-10 codes, and we also searched the charts of those with severe dystonia known to our clinical service. We included cases admitted to PICU from 2014 to 2024 who had dystonia severity grade 3-5, those with dystonia worse than their baseline, and with age greater than 30 days old, corrected for gestational age.
Results:
A total of 79 clinical records were screened; 41 admissions from 19 unique patients were included. Mean age was 7.6 ± 4.2 years; 53% were female. Most patients had a genetic etiology (n = 8, 42%). The presenting complaint per admission was often not dystonia (n = 24, 59%); infection was the most common trigger (n = 23, 56%) followed by pain (n = 6, 15%). Patients received several anti-dystonia medications (mean 6.9 ± 2.5), including clonidine, enteral or IV benzodiazepines, IV ketamine, and others. Median PICU stay was 7 days (interquartile range 3-14 days); 37% had multiple PICU admissions. Two patients (4.9%) died from status dystonicus complications.
Conclusions:
Status dystonicus is a life-threatening emergency commonly triggered by pain or infection in patients with dystonia. Given the considerable morbidity and mortality, multi-disciplinary teams should consider standardized treatment guidelines for these complex patients.
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