Accepted Manuscript Causes of failure of pallidal deep brain stimulation in cases with pre-operative diagnosis of isolated dystonia K. Amande M. Pauls, Joachim K. Krauss, Constanze E. Kämpfer, Andrea A. Kühn, Christoph Schrader, Martin Südmeyer, Niels Allert, Rainer Benecke, Christian Blahak, Jana K. Boller, Gereon R. Fink, Wolfgang Fogel, Thomas Liebig, Faycal El Majdoub, Philipp Mahlknecht, Josef Kessler, Joerg Mueller, Juergen Voges, Matthias Wittstock, Alexander Wolters, Mohammad Maarouf, Elena Moro, Jens Volkmann, Kailash P. Bhatia, Lars Timmermann PII:
S1353-8020(17)30230-4
DOI:
10.1016/j.parkreldis.2017.06.023
Reference:
PRD 3342
To appear in:
Parkinsonism and Related Disorders
Received Date: 9 December 2016 Revised Date:
22 May 2017
Accepted Date: 27 June 2017
Please cite this article as: Pauls KAM, Krauss JK, Kämpfer CE, Kühn AA, Schrader C, Südmeyer M, Allert N, Benecke R, Blahak C, Boller JK, Fink GR, Fogel W, Liebig T, El Majdoub F, Mahlknecht P, Kessler J, Mueller J, Voges J, Wittstock M, Wolters A, Maarouf M, Moro E, Volkmann J, Bhatia KP, Timmermann L, Causes of failure of pallidal deep brain stimulation in cases with pre-operative diagnosis of isolated dystonia, Parkinsonism and Related Disorders (2017), doi: 10.1016/j.parkreldis.2017.06.023. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Causes of failure of pallidal deep brain stimulation in cases with pre-operative diagnosis of isolated dystonia K Amande M Pauls1*, Joachim K Krauss2, Constanze E Kämpfer1, Andrea A Kühn3,
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Christoph Schrader4, Martin Südmeyer5, Niels Allert6, Rainer Benecke7, Christian Blahak8, Jana K Boller1, Gereon R Fink1,9, Wolfgang Fogel10, Thomas Liebig11, Faycal El Majdoub12, Philipp Mahlknecht13, Josef Kessler1, Joerg Mueller13,14, Juergen Voges15, Matthias
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Wittstock7, Alexander Wolters7, Mohammad Maarouf12, Elena Moro16, Jens Volkmann17, Kailash P Bhatia18, Lars Timmermann1
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1. Department of Neurology, Uniklinik Köln, Cologne, Germany
2. Department of Neurosurgery, Medizinische Hochschule Hannover, Hannover, Germany 3. Department of Neurology, Charite Universitätsmedizin Berlin, Berlin, Germany
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4. Department of Neurology, Medizinische Hochschule Hannover, Hannover, Germany 5. Department of Neurology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany
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6. Neurological Rehabilitation Center Godeshöhe, Bonn, Germany
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7. Department of Neurology, Universitätsmedizin Rostock, Rostock, Germany 8. Department of Neurology, Universitätsmedizin Mannheim, Mannheim, Germany 9. Institute of Neuroscience and Medicine (INM-3), Forschungszentrum Jülich, Jülich, Germany 10. Department of Neurology, Deutsche Klinik für Diagnostik, Wiesbaden, Germany 11. Department of Neuroradiology, Uniklinik Köln, Cologne, Germany
Pauls et al.
Failure of pallidal DBS in isolated dystonia
ACCEPTED MANUSCRIPT
12. Department of Stereotaxic and Functional Neurosurgery, Uniklinik Köln, Cologne, Germany 13. Department of Neurology, Medical University Innsbruck, Innsbruck, Austria
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14. Department of Neurology, Vivantes Klinikum Spandau, Berlin, Germany 15. Department for Stereotactic Neurosurgery, Universitätsklinikum Magdeburg, and Leibniz
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Institute of Neurobiology, Magdeburg, Germany
16. Division of Neurology, University Hospital Center of Grenoble, Grenoble, France
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17. Department of Neurology, Universitätsklinikum Würzburg, Würzburg, Germany 18. Institute of Neurology, University College London, London, UK *Corresponding author:
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Postal address: K. Amande M. Pauls, Department of Neurology, Uniklinik Köln, Kerpener Str. 62, 50924 Cologne, Germany. Phone: +49-221-478-84695, Fax: +49-221-478-97819
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Email:
[email protected]
Word count: abstract: 250 words, manuscript: 3063 words Key words: dystonia, deep brain stimulation (DBS), functional dystonia, neurodegenerative dystonia, pseudodystonia Funding The study was supported by the German Research Foundation (DFG grant KFO219). 2
Pauls et al.
Failure of pallidal DBS in isolated dystonia
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Abstract Introduction: Pallidal deep brain stimulation (GPi-DBS) is an effective therapy for isolated dystonia, but 10-20% of patients show improvement below 25-30%. We here investigated
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causes of insufficient response to GPi-DBS in isolated dystonia in a cross-sectional study. Methods: Patients with isolated dystonia at time of surgery, and C, p.V2716A het., c8578_8580delTCT, p.S2860del. het.)
tics*
suboptimal
misplaced
misplaced
misplaced
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** note that “other causes” includes patients which are formally nonresponders (according to scales or video), but are clinically improved upon re-examination
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suboptimal
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17
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16
misplaced
other disease
neurodegen. disease
fixed deformities
Legs and trunk: BFMDRS ON 48 points, BFMDRS OFF 48 points Rest of body: BFMDRS ON 14 points, BFMDRS OFF 24 points
electrode positioning, other disease
combined dystonia: dystonia and tics (tics remaining)
functional dystonia
clinically established functional
other disease
atypical Louis Bar syndrome
electrode positioning, other disease electrode positioning, other disease electrode positioning
other cause**
neurodegen. or acquired
neurodegen. or acquired significant improvement after electrode repositioning (BFMDRS-M before/after: 23/6 points) other cause: postoperativelvideo does not reflect postoperative patient status (patient is clinically better upon reexamination)
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ACCEPTED MANUSCRIPT
Impulse generator battery ACCEPTED MANUSCRIPT
ok
empty
Impedance check abnormal
Stimulation intensity
consider
low consider
ok
very low/high
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Side effect thresholds
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ok
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ok
Stimulation/ generator problem
consider
Lead misplacement/ dislocation
consider
consider
ok
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Lead location
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pathological
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normal Additional symptoms no
yes
Fixed deformities no
misplaced
borderline
Brain MRI
yes
both one
Neurodegenerative or acquired (lesionassociated) dystonia
consider other neurological signs movement disorder signs
functional signs
if extensive
Other causes or cause unknown
Combined dystonia Functional dystonia Fixed deformities
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We investigated causes for insufficient response of isolated dystonia to GPi-DBS Lead placement and stimulation issues are frequent and should be excluded first Non-isolated, functional and fixed dystonia account for most other cases These conditions should be looked for preoperatively to optimize counselling An assessment algorithm for insufficient GPi-DBS response is proposed
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