Deep brain stimulation in acute management of status dystonicus

The authors report the cases of 2 young male patients (aged 16 and 26 years) with dystonic cerebral palsy of unknown origin, who developed status dystonicus, an acute and persistent combination of generalized dystonia and chorea. Both patients developed status dystonicus after undergoing general anesthesia, and in 1 case, after administration of metoclopramide. In attempting to control this acute hyperkinetic movement disorder, multiple medication trials failed in both cases and patients required prolonged intubation and sedation with propofol. Bilateral deep brain stimulation of the globus pallidus internus (4 and 2 months after the onset of symptoms in the first and second case, respectively) produced immediate resolution of the hyperkinetic movement disorder in each case. Deep brain stimulation provided persistent suppression of the dystonic movement potential after a follow-up of 30 and 34 months, respectively, as demonstrated by the reemergence of severe dystonia during the end of battery life of the implantable pulse generators that was readily controlled by exchange of the generators in each case.

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Deep brain stimulation for medically refractory life-threatening status dystonicus in children

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.10.peds11360 ◽

2012 ◽

Vol 9 (1) ◽

pp. 99-102 ◽

Cited By ~ 25

Author(s):

Brian P. Walcott ◽

Brian V. Nahed ◽

Kristopher T. Kahle ◽

Ann-Christine Duhaime ◽

Nutan Sharma ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Symptom Control ◽

Respiratory Compromise ◽

Status Dystonicus ◽

Refractory Life ◽

Life Threatening ◽

Generalized Dystonia ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

Generalized dystonic syndromes may escalate into persistent episodes of generalized dystonia known as status dystonicus that can be life-threatening due to dystonia-induced rhabdomyolysis and/or respiratory compromise. Treatment of these conditions usually entails parenteral infusion of antispasmodic agents and sedatives and occasionally necessitates a medically induced coma for symptom control. The authors report a series of 3 children who presented with medically intractable, life-threatening status dystonicus and were successfully treated with bilateral pallidal deep brain stimulation. Bilateral globus pallidus internus stimulation appears to be effective in the urgent treatment of medically refractory and life-threatening movement disorders.

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Long-term effect of subthalamic and pallidal deep brain stimulation for status dystonicus in children with methylmalonic acidemia and GNAO1 mutation

Journal of Neural Transmission ◽

10.1007/s00702-019-02010-2 ◽

2019 ◽

Vol 126 (6) ◽

pp. 739-757 ◽

Cited By ~ 3

Author(s):

Alberto Benato ◽

Miryam Carecchio ◽

Alberto Burlina ◽

Francesco Paoloni ◽

Stefano Sartori ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Term Effect ◽

Methylmalonic Acidemia ◽

Long Term Effect ◽

Status Dystonicus ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

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Status Dystonicus Following Deep Brain Stimulation Surgery in DYT1 Dystonia Patients (P01.227)

Neurology ◽

10.1212/wnl.78.1_meetingabstracts.p01.227 ◽

2012 ◽

Vol 78 (Meeting Abstracts 1) ◽

pp. P01.227-P01.227 ◽

Cited By ~ 4

Author(s):

T. Cheung ◽

V. Flatow ◽

S. Ben-Haim ◽

I. Osborn ◽

C. Cho ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Dyt1 Dystonia ◽

Status Dystonicus ◽

Deep Brain Stimulation Surgery ◽

Deep Brain

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2.337 TREATMENT OF STATUS DYSTONICUS WITH PALLIDAL DEEP BRAIN STIMULATION — CASE REPORT

Parkinsonism & Related Disorders ◽

10.1016/s1353-8020(11)70660-5 ◽

2012 ◽

Vol 18 ◽

pp. S150

Author(s):

T. Mandat ◽

H. Koziara ◽

R. Rola ◽

T. Tykocki ◽

W. Bonicki ◽

...

Keyword(s):

Case Report ◽

Deep Brain Stimulation ◽

Brain Stimulation ◽

Status Dystonicus ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

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

Journal of Child Neurology ◽

10.1177/0883073818756134 ◽

2018 ◽

Vol 33 (6) ◽

pp. 413-416 ◽

Cited By ~ 11

Author(s):

C. Michael Honey ◽

Armaan K. Malhotra ◽

Maja Tarailo-Graovac ◽

Clara D. M. van Karnebeek ◽

Gabriella Horvath ◽

...

Keyword(s):

Intensive Care Unit ◽

Intensive Care ◽

Deep Brain Stimulation ◽

Intensive Care Unit Admission ◽

Pediatric Intensive Care Unit ◽

Brain Stimulation ◽

De Novo ◽

Pediatric Intensive Care ◽

Status Dystonicus ◽

Deep Brain

Dystonic storm or status dystonicus is a life-threatening hyperkinetic movement disorder with biochemical alterations due to the excessive muscle contractions. The medical management can require pediatric intensive care unit admission and a combination of medications while the underlying trigger is managed. Severe cases may require general anesthesia and paralytic agents with intubation and may relapse when these drugs are weaned. Deep brain stimulation of the globus pallidum has been reported to terminate dystonic storm in several pediatric cases. We present a 10-year-old boy with a de novo GNAO1 mutation–induced dystonic storm who required a 2-month pediatric intensive care unit admission and remained refractory to all medical treatments. Deep brain stimulation was performed under general anesthetic without complication. His dyskinetic movements stopped with initiation of stimulation. He was discharged from the pediatric intensive care unit after 4 days. We present prospectively evaluated changes in dystonia symptoms and quality of life for a patient with GNAO1 mutation treated with deep brain stimulation.

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