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Deep brain stimulation in a young child with GNAO1 mutation - Feasible and helpful
Eva Lai-Wah Fung1, Chung-Yin Mo2, Sharon Tsui-Hang Fung2
1Department of Paediatrics, The Chinese University of Hong Kong, Hong Kong.
Insights
GNAO1-related movement disorders in children can be severe. Deep brain stimulation (DBS) offers a promising treatment option, even for young patients, improving outcomes after acute exacerbations.
Area of Science:
- Neurology
- Genetics
- Pediatric Neurology
Background:
- GNAO1-related disorder presents with hypotonia, developmental delay, epilepsy, and movement disorders, posing life-threatening risks during exacerbations.
- Deep Brain Stimulation (DBS) is approved in the USA for treatment-resistant primary dystonia in children aged 7 and older.
Observation:
- A 4-year-old girl with GNAO1-related dyskinesia and global developmental delay experienced severe, infection-triggered dyskinesia requiring intensive care.
- The patient showed dramatic improvement in dyskinesia following DBS implantation.
- Technical challenges and safety considerations for DBS in preschool-aged children were examined.
Findings:
- Deep brain stimulation (DBS) can be highly effective in managing severe GNAO1-related movement disorders in young children.
- Early consideration of DBS, particularly after dyskinetic crises, is recommended due to the potential for recurrence.
Implications:
- DBS should be considered as an early treatment option for drug-resistant movement disorders in young children with GNAO1 mutations.
- Comprehensive presurgical counseling and vigilant post-operative monitoring are crucial for managing DBS in pediatric patients.
Background:
GNAO1 is an emerging disorder characterized with hypotonia, developmental delay, epilepsy, and movement disorder, which can be potentially life threatening during acute exacerbation. In the USA, deep brain stimulation (DBS) has been licensed for treating children with chronic, treatment-resistant primary dystonia, who are 7 years old or older.
Case Description:
A 4-year-old girl diagnosed to have GNAO1-related dyskinesia and severe global developmental delay. She had severe dyskinesia precipitated by intercurrent infection, requiring prolonged intensive care for heavy sedation and related complications. Her dyskinesia improved dramatically after DBS implantation. Technical difficulties and precautions of DBS in preschool children were discussed.
Conclusion:
DBS should be considered early in the treatment of drug-resistant movement disorders in young children with GNAO1, especially after dyskinetic crisis, as they tend to recur. Presurgical counseling to parents and close monitoring of complications is also important in the process.
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