Neuropediatrics 2013; 44 - FV15_07
DOI: 10.1055/s-0033-1337744

Young adults with dyskinetic cerebral palsy improve subjectively on pallidal stimulation but not in formal dystonia, gait, speech, and swallowing testing

A Koy 1, A Pauls 1, P Flossdorf 1, J Becker 1, E Schönau 2, M Maarouf 3, T Liebig 4, O Fricke 2, GR Fink 1, L Timmermann 1
  • 1Klinik für Neurologie, Universitätsklinikum, Köln, Germany
  • 2Klinik für Kinder-und Jugendmedizin, Universitätsklinik, Köln, Germany
  • 3Klinik für Funktionelle und Stereotaktische Neurochirurgie, Universitätsklinik Köln, Köln, Germany
  • 4Klinik für Diagnostische Radiologie, Universitätsklinik, Köln, Germany

Aims: Cerebral palsy (CP) is a common cause for secondary dystonia. Pharmacological treatment is often unsatisfactory or side effects are dose limiting. There is only limited data about the effect of deep brain stimulation (DBS) in patients with dyskinetic CP. A meta-analysis on the percentage change in the Burke-Fahn-Marsden dystonia rating scale (BFMDRS) revealed an improvement of 23% in patients with dyskinetic CP and DBS. In this study, we assessed the effect of pallidal DBS on dystonia, gait, speech, swallowing, and subjective perception of symptoms.

Methods: A total of 8 adult CP patients who underwent DBS of the Globus Pallidus internus were investigated. BFMDRS was compared pre- and postoperatively in a retrospective approach. Speech (oral diachokinesia, Frenchay Dysarthria test), swallowing (fiberoptic laryngoscopy), and gait (Leonardo Mechanography) were analyzed under chronic therapeutic stimulation (ON) and after the stimulation had been switched off (OFF). Pre- and postoperative videos were rated by a blinded neurologist (K.A.M.P.). The patients were asked for their subjective perception of symptoms by a Clinical Global Impression (CGI scale 0 to 10).

Results: The mean preoperative BFMDRS was 64.5 ± 38.7 compared with 65.1 ± 40.3 postoperatively. Comparing ON and OFF, there were no significant acute changes. With respect to gait path length/distance increased during OFF, other gait parameters as well as swallowing did not change significantly. Oral diadochokinesia tasks deteriorated mildly during ON. However, the mean improvement according to patients' CGI was 3.3 out of 10.

Conclusion: In our cohort, patients with dyskinetic CP seem to improve subjectively under pallidal stimulation but without measurable changes in formal testing for dystonia, gait, swallowing, and speech. However, CP patients classified as nonresponders according to the BFMDRS nevertheless frequently report subjective improvement of symptoms after DBS. A comprehensive assessment of motor function, speech, swallowing, and quality of life in a larger cohort of CP patients may help to quantify the overall effect of DBS more thoroughly.