Objective: Status dystonicus, also known as dystonic storm or dystonic crisis, is a life-threatening movement disorder emergency. We report pediatric status dystonicus from a tertiary care center in North India.

Methods: Retrospective review of case-sheets of children admitted with a diagnosis of ‘dystonic storm’ or ‘status dystonicus’. All demographic and clinical details were recorded in a pre-structured proforma and analyzed.

Results: Twenty consecutive children with dystonic storm/status dystonicus were admitted over the past 4 years in the Department of Pediatrics. Of these, majority (70%) were males. Mean age was 7 years (age range 1 to 11 years). Common underlying diagnoses included cerebral palsy, neurowilson, mitochondrial cytopathy, and pantothenate kinase-associated neurodegeneration. All children had generalized dystonia. Triggering factors identified were intercurrent illness/infection, rapid tapering of benzodiazepines and inadvertent withdrawal of zinc therapy in Wilson disease. Majority of the patients (80%) had complications as follows: respiratory (50%) requiring oxygen support, feeding problems (50%) requiring nasogastric feeding, rhabdomyolysis (30%), renal function derangement (30%) dehydration (30%) and fever (20%). Medications commonly used were intravenous lorazepam (10%), oral chloral hydrate (10%), intravenous midazolam (60%), oral levodopa (20%), oral trihexyphenydyl in all, oral tetrabenazine (50%), intravenous clonidine (40%), oral benzodiazepines (70%), oral baclofen (80%), oral gabapentin (10%) and oral melatonin (10%). All children had sequelae in follow-up.

Conclusion: Although rare, status dystonicus is a movement disorder emergency that requires prompt diagnosis and therapeutic interventions, identification of the underlying cause and trigger, and management of associated complications including metabolic, renal and ventilatory complications.