Neuropediatrics 2024; 55(S 01): S1-S25
DOI: 10.1055/s-0044-1791887
Bewegungsstörungen

Effects of GPi-DBS on Speech and Swallowing in Pediatric Patients with Dystonia

K. Bernardi
1   Department of Pediatrics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
,
A. A. Kühn
3   Department of Neurology, Charité University Medicine Berlin, Berlin, Germany
,
A. Marcelino
3   Department of Neurology, Charité University Medicine Berlin, Berlin, Germany
,
M. Eckenweiler
4   Department of Neuropediatrics and Muscle Disorders, University Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany
,
C. Rensing-Zimmermann
4   Department of Neuropediatrics and Muscle Disorders, University Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany
,
J. K. Krauss
5   Department of Neurosurgery, Hannover Medical School, Hannover, Germany
,
J. Runge
5   Department of Neurosurgery, Hannover Medical School, Hannover, Germany
,
D. Lorenz
6   Department of Pediatrics, University Children’s Hospital, Wuerzburg, Germany
,
M. Müller
6   Department of Pediatrics, University Children’s Hospital, Wuerzburg, Germany
,
A. Schnitzler
7   Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
,
A. Bevot
9   Department of Pediatric Neurology and Developmental Medicine, University Children’s Hospital Tübingen, Tübingen, Germany
,
V. Visser-Vandewalle
10   Department of Stereotactic and Functional Neurosurgery, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
,
L. Timmermann
11   Department of Neurology, University Hospital of Marburg, Marburg, Germany
,
P. Schiller
12   Institute of Medical Statistics and Computational Biology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
,
A. Koy
1   Department of Pediatrics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
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Background/Purpose: Bilateral pallidal deep brain stimulation (GPi-DBS) has been proven as a safe and effective treatment in certain forms of inherited dystonia (ID), and its effects in acquired dystonia such as in dyskinetic cerebral palsy (DCP) vary widely. Swallowing and speech significantly affect quality of life of these patients, yet the impact of GPi-DBS on these domains, especially in pediatric patients, is not well understood. This study evaluates GPi-DBS effects on speech and swallowing using the Frenchay Dysarthria Assessment 2 (FDA-2), in pediatric patients with dystonia.

Methods: This retrospective multicentric study analyzes speech and swallowing pre- and 12 months post-GPi-DBS using FDA-2, including data from the STIM-CP trial (16-03) and the GEPESTIM registry (DRKS813-168). FDA-2 comprises seven sections: reflexes, respiration, lips, palate, laryngeal, tongue, and intelligibility. Ratings range from normal to severe abnormal with total score of 104 indicating optimal performance.

Results: A total of 26 patients were included (17 m; mean age at DBS: 12.2 years); 14 with DCP, and 12 with ID. The FDA-2 total scores were significantly higher in ID than DCP patients pre-DBS (median, 25th–75th percentile: DCP 21.5, 14–39, ID 58.5, 41–103; p = 0.005, Kruskal–Wallis test) and post-DBS (mean DCP 31 vs. mean ID 67; median DCP 21.8, 16–43 vs. median ID 64.5, 37–104; p = 0.007). There were no significant changes in FDA-2 scores pre- and post-DBS for all patients; median scores remained unchanged. However, small improvements in reflexes, lips, and tongue sections were observed in ID patients (nonsignificant). Further modeling will be done.

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.



Publication History

Article published online:
08 October 2024

Georg Thieme Verlag KG
Stuttgart · New York