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Pediatric Dystonic Storm: A Hospital-Based Study
Jyotindra Narayan Goswami1, Shuvendu Roy1, Saroj Kumar Patnaik1
1Department of Pediatrics, Army Hospital (R&R), Delhi, India.
Insights
Pediatric dystonic storm is a serious medical emergency requiring prompt, multi-faceted treatment. Aggressive management, including supportive care and medications, helps control symptoms and prevent complications in children.
Area of Science:
- Pediatric Neurology
- Movement Disorders
Background:
- Pediatric dystonic storm is an underrecognized neurological emergency.
- It presents as severe, sustained muscle contractions causing significant distress and functional impairment in children.
Purpose of the Study:
- To evaluate the clinical profiles of children experiencing dystonic storm.
- To analyze management strategies and treatment responses in this pediatric population.
Main Methods:
- A retrospective review was conducted on children (up to 18 years) hospitalized with dystonic storm over 39 months.
- Data collected included demographics, underlying conditions, triggers, medications, interventions, and outcomes.
Main Results:
- Twenty-three children (median age: 6 years 11 months) presented with dystonic storm, with an annual incidence of 0.4 per 1,000 admissions.
- Most children required polypharmacotherapy and supportive care, with midazolam infusion and mechanical ventilation used in severe cases.
- While no deaths occurred, rhabdomyolysis was noted in one child, and 26% experienced post-discharge relapses.
Conclusions:
- Dystonic storm is a medical emergency requiring aggressive, multimodal management.
- Supportive care, antidystonic medications, and early elective ventilation with adequate sedation are crucial for ameliorating complications.
- Relapses of dystonic storm are common and necessitate ongoing monitoring.
Objective:
Pediatric dystonic storm is an underrecognized entity. We aimed to evaluate the profiles of children presenting with dystonic storm in a referral hospital. Management schema and treatment responsiveness of this uncommonly reported entity were analyzed.
Methods:
Retrospective review of all children (up to 18 years) hospitalized with dystonic storm over 39 months in the aforementioned facility.
Results:
Twenty-three children whose ages ranged from 2 years 2 months to 14 years 4 months years (median: 6 years 11 months) (males: 13, females: 11) presented with dystonic storm. The annual incidence was 0.4 per 1,000 fresh admissions with an event rate of 0.9 per 1,000 for all admissions. All had Dystonia Severity Action Plan grades 4/5 with identifiable trigger in 13 (50%). Underlying dystonic disorder preexisted in 10 (43.4%); 8 of these had cerebral palsy. Polypharmacotherapy with >4 drugs out of trihexyphenidyl, tetrabenazine, clonazepam, gabapentin, levodopa-carbidopa, trichlorophos, and melatonin was needed. Supportive care and adequate sedation helped in symptom control. All children were managed with midazolam infusion over 2-10 days (median: 5 days). Mechanical ventilation was resorted to in 6 children (3-22 days). Vecuronium and propofol were used in 3/23 (13%) and 4/23 (17%) children, respectively. Deep brain stimulation was curative in 1 child. Hospitalization ranged from 5 to 31 (median: 11) days. Although there were no deaths, rhabdomyolysis was noted in 1 child. Postdischarge, 6 (26%) children relapsed.
Conclusions:
Dystonic storm is a medical emergency mandating aggressive multimodal management. Supportive care, antidystonic drugs, and early elective ventilation alongside adequate sedation with benzodiazepines ameliorate complications. Relapses of dystonic storm are not uncommon.
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