Mov Disord Clin Pract. 2025 Nov 27. doi: 10.1002/mdc3.70454. Online ahead of print.
ABSTRACT
BACKGROUND: Bilateral globus pallidus internus deep brain stimulation (GPi-DBS) is a proven safe and effective treatment in certain forms of idiopathic or inherited dystonia (ID/IN). Its effects in acquired dystonia such as in dyskinetic cerebral palsy (DCP) however vary widely. The impact of GPi-DBS on speech and swallowing, which significantly affect quality of life, remains poorly understood, especially in pediatric patients.
OBJECTIVE: To evaluate GPi-DBS effects on speech and swallowing using the Frenchay Dysarthria Assessment 2 (FDA-2), in pediatric patients with dystonia, and assess how the effects differ between DCP and ID/IN patients.
METHODS: This pro- and retrospective multicenter study analyzes speech and swallowing pre- and 12 months post-GPi-DBS using FDA-2, including prospective data from the STIM-CP trial and retrospective data from the GEPESTIM registry.
RESULTS: Twenty-six patients were included (17 male, 9 female; 14 DCP, 12 ID/IN) with mean age of 12.2 years at DBS. No significant changes in FDA-2 total scores were observed pre- and post-DBS (pre: 46.3 ± 33.6; post: 46.3 ± 34.2). ID/IN patients showed consistently higher scores compared to DCP patients both pre- and post-DBS (P < 0.005). When adjusted for age, medication, and pre-surgical values, group differences narrowed, with minimal changes from baseline in both groups.
CONCLUSION: GPi-DBS did not significantly change FDA-2 scores pre- and post-DBS. Assessing speech and swallowing in pediatric patients with dystonia, impaired expressive language and/or intellectual disability is challenging. More comprehensive and patient-centered assessment tools are needed to fully capture DBS effects on these domains in these complex disabled patients.
PMID:41307194 | DOI:10.1002/mdc3.70454
