Movement disorders clinical practice · 2025
Pediatric Status Dystonicus: A Single-Center 10-Year Retrospective Review.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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Peacock Dakota J S J, Vithlani Keshvi, Clarke Grace A, Patel Mona, Joseph Akshaya Ann, Horvath Gabriella
Аннотация
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.
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.