Pediatric Dystonic Storm

Abstract

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.

Case Report: Dystonic Storm Following Japanese Encephalitis Virus Infection

“…Dystonic storm (also called status dystonicus) is a neurological emergency characterized by sustained/intermittent involuntary generalized muscle contractions resulting in repetitive painful twisting movements and abnormal postures. [1][2][3][4][5][6] Although it typically occurs in patients with diagnosed primary dystonic syndromes (i.e., DYT-1 mutation-associated dystonia), it can be seen in secondary dystonic states (i.e., patients with inborn errors of metabolism, dystonic cerebral palsy, Wilson's disease, pantothenate kinase-associated neurodegeneration, and exposure to drugs, among others). [1][2][3][4][5][6] Treatment of this neurological emergency is demanding and requires admission to the intensive care unit.…”

“…[1][2][3][4][5][6] Although it typically occurs in patients with diagnosed primary dystonic syndromes (i.e., DYT-1 mutation-associated dystonia), it can be seen in secondary dystonic states (i.e., patients with inborn errors of metabolism, dystonic cerebral palsy, Wilson's disease, pantothenate kinase-associated neurodegeneration, and exposure to drugs, among others). [1][2][3][4][5][6] Treatment of this neurological emergency is demanding and requires admission to the intensive care unit. [1][2][3][4][5][6] Stoppage of inciting agents/drugs and prescription of anticholinergics, benzodiazepines, baclofen, and dopamine depletors remain the mainstay of medical management.…”

“…[1][2][3][4][5][6] Treatment of this neurological emergency is demanding and requires admission to the intensive care unit. [1][2][3][4][5][6] Stoppage of inciting agents/drugs and prescription of anticholinergics, benzodiazepines, baclofen, and dopamine depletors remain the mainstay of medical management. [1][2][3][4][5][6] Mechanical ventilation for several days to weeks with intravenous sedation, airway protection, and paralysis have often been the ultimate need to resolve the crisis.…”

“…[1][2][3][4][5][6] Stoppage of inciting agents/drugs and prescription of anticholinergics, benzodiazepines, baclofen, and dopamine depletors remain the mainstay of medical management. [1][2][3][4][5][6] Mechanical ventilation for several days to weeks with intravenous sedation, airway protection, and paralysis have often been the ultimate need to resolve the crisis. [1][2][3][4][5][6] Even with the best possible management, the mortality rate is nearly 10%.…”

“…[1][2][3][4][5][6] Mechanical ventilation for several days to weeks with intravenous sedation, airway protection, and paralysis have often been the ultimate need to resolve the crisis. [1][2][3][4][5][6] Even with the best possible management, the mortality rate is nearly 10%. 5 And even in cases of survival, recovery is seldom complete, and residual deficits and relapses are frequent.…”