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.

Keywords:
Deep brain stimulation, Burke-Fahn-Marsden Dystonia Rating Scale
1.
Treves T, Korczyn AD: Progressive dystonia and paraparesis in cerebral palsy. Eur Neurol 1986;25:148-153.
2.
Jankovic J: Treatment of dystonia. Lancet Neurol 2006;5:864-872.
3.
Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Lagrange C, Yelnik J, et al; French SPIDY Study Group: Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 2007;6:223-229.
4.
Kupsch A, Benecke R, Müller J, Trottenberg T, Schneider GH, Poewe W, et al; Deep-Brain Stimulation for Dystonia Study Group: Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 2006;355:1978-1990.
5.
Marks WA, Honeycutt J, Acosta F Jr, Reed M, Bailey L, Pomykal A, Mercer M: Dystonia due to cerebral palsy responds to deep brain stimulation of the globus pallidus internus. Mov Disord 2011;26:1748-1751.
6.
Vidailhet M, Yelnik J, Lagrange C, Fraix V, Grabli D, Thobois S, et al; French SPIDY-2 Study Group: Bilateral pallidal deep brain stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study. Lancet Neurol 2009;8:709-717.
7.
Gimeno H, Tustin K, Selway R, Lin JP: Beyond the Burke-Fahn-Marsden Dystonia Rating Scale: deep brain stimulation in childhood secondary dystonia. Eur J Paediatr Neurol 2012;16:501-508.
8.
Koy A, Hellmich M, Pauls KA, Marks W, Lin JP, Fricke O, Timmermann L: Effects of deep brain stimulation in dyskinetic cerebral palsy: a meta-analysis. Mov Disord 2013;28:647-654.
9.
Jahnsen R, Aamodt G, Rosenbaum P: Gross Motor Function Classification System used in adults with cerebral palsy: agreement of self-reported versus professional rating. Dev Med Child Neurol 2006;48:734-738.
10.
Burke RE, Fahn S, Marsden CD, Bressman SB, Moskowitz C, Friedman J: Validity and reliability of a rating scale for the primary torsion dystonia. Neurology 1985;35:73-77.
11.
Munetz MR, Benjamin S: How to examine patients using the abnormal involuntary movement scale. Hosp Community Psychiatry 1988;39:1172-1177.
12.
Veilleux LN, Robert M, Ballaz L, Lemay M, Rauch F: Gait analysis using a force-measuring gangway: intrasession repeatability in healthy adults. J Musculoskelet Neuronal Interact 2011;11:27-33.
13.
Enderby P: Frenchay Dysarthria Assessment. Int J Lang Commun Disord 1980;15:165-173.
14.
Duffy JR: Motor Speech Disorders: Substrates, Differential Diagnosis, and Management, ed 2. St Louis: Elsevier Mosby, 2003.
15.
Ansel BM, Kent RD: Acoustic-phonetic contrasts and intelligibility in the dysarthria associated with mixed cerebral palsy. J Speech Hear Res 1992;35:296-308.
16.
Thoonen G, Maassen B, Wit J, Gabreels F, Schreuder R: The integrated use of maximum performance tasks in differential diagnosis evaluations among children with motor speech disorders. Clin Linguist Phon 1996;10:311-336.
17.
Leder SB, Espinosa JF: Aspiration risk after acute stroke: comparison of clinical examination and fiberoptic endoscopic evaluation of swallowing. Dysphagia 2002;17:214-218.
18.
Dziewas R, Warnecke T, Olenberg S, Teismann I, Zimmermann J, Kramer C, Ritter M, Ringelstein EB, Schabitz WR: Towards a basic endoscopic assessment of swallowing in acute stroke - development and evaluation of a simple dysphagia score. Cerebrovasc Dis 2008;26:41-47.
19.
Schaltenbrand G, Wahren A: Atlas for stereotaxy of the human brain, ed 3. Stuttgart, Thieme, 1977.
20.
Katsakiori PF, Kefalopoulou Z, Markaki E, Paschali A, Ellul J, Kagadis GC, et al: Deep brain stimulation for secondary dystonia: results in 8 patients. Acta Neurochir (Wien) 2009;151:473-478.
21.
Vercueil L, Pollak P, Fraix V, Caputo E, Moro E, Benazzouz A, et al: Deep brain stimulation in the treatment of severe dystonia. J Neurol 2001;248:695-700.
22.
Pretto TE, Dalvi A, Kang UJ, Penn RD: A prospective blinded evaluation of deep brain stimulation for the treatment of secondary dystonia and primary torticollis syndromes. J Neurosurg 2008;109:405-409.
23.
Tronnier VM, Fogel W: Pallidal stimulation for generalized dystonia. Report of three cases. J Neurosurg 2000;92:453-456.
24.
Isaias IU, Alterman RL, Tagliati M: Outcome predictors of pallidal stimulation in patients with primary dystonia: the role of disease duration. Brain 2008;131:1895-1902.
25.
Andrews C, Aviles-Olmos I, Hariz M, Foltynie T: Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes. J Neurol Neurosurg Psychiatry 2010;81:1383-1389.
26.
Speelman JD, Contarino MF, Schuurman PR, Tijssen MA, de Bie RM: Deep brain stimulation for dystonia: patient selection and outcomes. Eur J Neurol 2010;17(suppl 1):102-106.
27.
Cramer SC, Sur M, Dobkin BH, O'Brien C, Sanger TD, Trojanowski JQ, Rumsey JM, et al: Harnessing neuroplasticity for clinical applications. Brain 2011;134:1591-1609.
28.
Yokochi K: Clinical profiles of children with cerebral palsy having lesions of the thalamus, putamen and/or peri-Rolandic area. Brain Dev 2004;26:227-232.
29.
Menkes JH, Curran J: Clinical and MR correlates in children with extrapyramidal cerebral palsy. AJNR Am J Neurorad 1994;15:451-457.
30.
Schrader C, Capelle HH, Kinfe TM, Blahak C, Bäzner H, Lütjens G, Dressler D, Krauss JK: GPi-DBS may induce a hypokinetic gait disorder with freezing of gait in patients with dystonia. Neurology 2011;77:483-488.
31.
Fasano A, Daniele A, Albanese A: Treatment of motor and non-motor features of Parkinson's disease with deep brain stimulation. Lancet Neurol 2012;11:429-442.
© 2014 S. Karger AG, Basel
2014
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.