Pediatric dystonic storm : A hospital-based study

Background: Pediatric dystonic storm is an under-recognized 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: 23 children aged 2year 2months to 14 year 4 months years (median:6 year 11 months)(males: 13, females:11) presented with dystonic storm. Annual incidence was 0.4 per 1000 fresh admissions with event rate of 0.9 per 1000 for all admissions. All had Dystonia Severity Action Plan (DSAP) grades 4/5 with identifiable trigger in 13 (50%). Underlying dystonic disorder pre-existed in 10(43.4%); 08 of these had Cerebral Palsy . Polypharmacotherapy with >4 drugs out of trihexyphenydyl, tetrabenazine, clonazepam, gabapentin, levodopa-carbidopa, triclophos and melatonin were 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 5-31(median: 11) days. While there were no deaths, rhabdomyolysis was noted in 1 child. Post discharge,6(26%) children relapsed.

Conclusions: Dystonic storm is a medical emergency mandating aggressive multimodal management. Supportive care, anti-dystonic drugs, early elective ventilation alongside adequate sedation with benzodiazepines ameliorate complications. Relapses of dystonic storm are not uncommon.