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Pallidal deep brain stimulation in a 5-year-old child with dystonic storm: case report
Sabri Aydin1, Bashar Abuzayed, Serap Uysal
1Istanbul University, Cerrahpasa Faculty of Medicine, Department of Neurosurgery, Istanbul, Turkey.
Insights
Deep brain stimulation (DBS) offers a life-saving treatment for severe dystonia in children. This case study highlights successful DBS in a 5-year-old, significantly reducing dystonia symptoms.
Area of Science:
- Neurology
- Neurosurgery
- Pediatrics
Background:
- Epilepsy and mental retardation can be associated with severe movement disorders.
- Dystonic storm is a life-threatening condition characterized by severe, prolonged muscle contractions.
Observation:
- A 5-year-old child presented with a life-threatening dystonic storm, bilateral pallidal calcification, and a history of epilepsy and mental retardation.
- Conventional treatments failed to alleviate the patient's severe dystonic symptoms.
Findings:
- Deep brain stimulation (DBS) targeting the bilateral globus pallidus internus (GPi) was surgically implanted.
- The patient experienced an 81% reduction in dystonia severity 15 months post-surgery.
- This represents the youngest patient reported in literature to undergo DBS for dystonia.
Implications:
- DBS is a viable and effective treatment option for severe pediatric dystonia, even in very young patients.
- This case demonstrates the potential of DBS as a life-saving intervention for refractory dystonia.
- Further research into early DBS intervention for pediatric movement disorders is warranted.
Abstract:
A 5-year-old child had a medical history of epilepsy and a newly presented mental retardation with a life-threatening dystonic storm. Neuroimagings showed bilateral calcification of the pallidum. Several treatment modalities were performed, but the symptoms showed no significant improvement. The patient was operated on in order to place a deep brain stimulation (DBS) targeting bilateral globus pallidum internus (GPi). The dystonia showed a remarkable improvement after surgery, with 81% reduction of dystonia severity after 15 months. To our best knowledge, this is the youngest patient mentioned in the literature to be treated with DBS, which was also life-saving in this case.
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