To quantify the number of admissions involving status dystonicus (SD) in a large pediatric hospital. Secondary objectives were to identify the etiology of dystonia and triggers of SD, and evaluate key outcomes including transfer to ICU and complications of SD. 

Dystonia is a common movement disorder in children with neurological impairment, manifesting as repetitive twisting/abnormal postures. Various triggers (stress, pain, infections) can exacerbate dystonia. SD is the most extreme and life-threatening form of dystonia. As part of an ongoing quality improvement project, we analyzed the prevalence and outcomes of SD over the past 4.5 years at our institution. 

We performed a retrospective analysis of electronic medical records for patients with dystonia admitted to The Hospital for Sick Children from January 2020 to June 2024.  We only included children admitted with SD and excluded elective admissions. 

Over 4.5 years, there were 92 admissions for 48 unique patients with SD, an average of 20/year. Of these, 45%(41/92) had genetic/metabolic dystonia and 42%(39/92) had acquired dystonia.  A trigger for worsening dystonia was identified in 82%(75/92) of admissions, especially in children with acquired dystonia (35/39, 90%).  

Systemic complications of SD (rhabdomyolysis, respiratory dysfunction, fractures) were observed in 39%(36/92). ICU care was required in 22%(21/92) of admissions. Nine admissions resulted in emergent deep brain stimulation (DBS) for SD (12.5%), while 20 children (21.7%) had already received DBS prior to admission. Two deaths (2/48) occurred, both in patients with a genetic diagnosis. 

SD is common in pediatric hospitals and contributes to morbidity and mortality. Triggers were identified in most cases, stressing the need to assess triggers in all SD admissions. SD can occur in any child with dystonia, regardless of diagnosis, and often recurs in the same patient. SD can lead to complications or death without timely intervention, highlighting the need for appropriate treatments.