Deep brain stimulation in a young child with GNAO1 mutation - Feasible and helpful.

Autor: Fung EL; Department of Paediatrics, The Chinese University of Hong Kong, Hong Kong., Mo CY; Department of Paediatrics, Kwong Wah Hospital, Hong Kong., Fung ST; Department of Paediatrics, Kwong Wah Hospital, Hong Kong., Chan AY; Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong., Lau KY; Department of Surgery, Division of Neurosurgery, The Chinese University of Hong Kong, Hong Kong., Chan EK; Department of Surgery, Division of Neurosurgery, The Chinese University of Hong Kong, Hong Kong., Chan DY; Department of Surgery, Division of Neurosurgery, The Chinese University of Hong Kong, Hong Kong., Zhu XL; Department of Surgery, Division of Neurosurgery, The Chinese University of Hong Kong, Hong Kong., Chan DT; Department of Surgery, Division of Neurosurgery, The Chinese University of Hong Kong, Hong Kong., Poon WS; Department of Surgery, Division of Neurosurgery, The Chinese University of Hong Kong, Hong Kong.
Jazyk: angličtina
Zdroj: Surgical neurology international [Surg Neurol Int] 2022 Jul 01; Vol. 13, pp. 285. Date of Electronic Publication: 2022 Jul 01 (Print Publication: 2022).
DOI: 10.25259/SNI_166_2022
Abstrakt: 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.
Competing Interests: There are no conflicts of interest.
(Copyright: © 2022 Surgical Neurology International.)
Databáze: MEDLINE