Supplementary Material for: Young Adults with Dyskinetic Cerebral Palsy Improve Subjectively on Pallidal Stimulation, but not in Formal Dystonia, Gait, Speech and Swallowing Testing

journal contribution
posted on 09.03.2017 by Koy A., Pauls K.A.M., Flossdorf P., Becker J., Schönau E., Maarouf M., Liebig T., Fricke O., Fink G.R., Timmermann L.

Background: Pharmacological treatment of dyskinetic cerebral palsy (CP) is often ineffective. Data about outcome of deep brain stimulation (DBS) in these patients remains scarce. Methods: Eight patients with dyskinetic CP and DBS of the Globus Pallidus internus were investigated. Using pre- and postoperative videos the severity of dystonia and changes thereof during standardized settings (‘on') and after the stimulator had been switched off (‘off') were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Furthermore, subjective impression (SI) of the extent of postoperative change as well as gait (Leonardo Mechanograph® Gangway), speech (Frenchay Dysarthria) and swallowing performances (fiberoptic laryngoscopy) were assessed during ‘on' and ‘off'. Results: When comparing pre- and postoperative as well as ‘on' and ‘off', the BFMDRS and most of the gait, speech, and swallowing parameters did not differ significantly. In contrast, patients reported significant improvement of their SI postoperatively (3.1 on a 10-point-scale). Conclusion: Data show that our CP-patients did not benefit from GPi-DBS when tested formally for dystonia, gait, speech and swallowing. In stark contrast, these patients reported significant subjective improvement. Taken together, and in light of current unsatisfactory medical treatment options, our data suggest that further assessment of the effects of GPi-DBS in dyskinetic CP is warranted.