ICNC Abstracts, ICNC 2018

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Status dystonicus in Children: A Nightmare for both the Physician and the Patient
Arushi Gahlot Saini, Kanika Goyal, Sumeet Dhawan, Lokesh Saini, Renu Suthar, Jitendra Sahu, Naveen Sakhyan, Jayashree Muralidharan, Pratibha Singhi

Last modified: 2018-09-09

Abstract


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.


Keywords


Status dystonicus; Movement disorder emergency; Children; Dystonic crisis

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