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GNAO1 Mutation-Induced Pediatric Dystonic Storm Rescue With Pallidal Deep Brain Stimulation

C Michael Honey1, Armaan K Malhotra2, Maja Tarailo-Graovac3,4,5,6

  • 11 Department of Surgery, Section of Neurosurgery, University of Manitoba, Winnipeg, Canada.

Journal of Child Neurology
|April 18, 2018
PubMed

Insights

Dystonic storm, a severe movement disorder, can be effectively treated with deep brain stimulation. This therapy rapidly resolved symptoms in a pediatric patient with a GNAO1 mutation, offering a new treatment avenue.

Area of Science:

  • Neurology
  • Genetics
  • Pediatric Intensive Care

Background:

  • Dystonic storm (status dystonicus) is a critical hyperkinetic movement disorder causing excessive muscle contractions and biochemical changes.
  • Management often involves pediatric intensive care unit (PICU) admission, medication, and sometimes general anesthesia with intubation for severe cases.
  • Deep brain stimulation (DBS) of the globus pallidus has shown promise in resolving dystonic storms in pediatric patients.

Keywords:
GNAO1 mutationdeep brain stimulationstatus dystonicus

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