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Pallidal deep brain stimulation in juvenile Huntington's disease: local field potential oscillations and clinical data

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Abstract

Background

Recently, therapeutic attempts to control motor choreatic hyperkinesia of Huntington’s disease (HD) by means of pallidal deep brain stimulation (Gp-DBS) were successful. With respect to the clinical effects of Gp-DBS in juvenile hypokinetic-rigid HD (jHD; Westphal variant), only one single-case has been reported up to date. Oscillatory patterns of the Gp in jHD are not known.

Objectives and methods

This work aimed to analyse pallidal local field potential oscillations (LFP) in two patients with jHD treated with Gp-DBS. Safety data and clinical scores up to 12 months after DBS-electrode implantation were collected in the framework of a prospective trial (ClinicalTrials.gov; NCT00902889).

Results

Intraoperative LFP revealed local alpha and beta oscillations similar to those found in other movement disorders with akinetic rigid and dystonic presentation. Significant motor improvement was not found. There were no treatment-related complications or unresolved long-term adverse events.

Conclusions

In spite of similar intraoperative LFP patterns of jHD with those of movement disorders benefitting from DBS, clinical results were not convincing in our patients, so that Gp-DBS in jHD cannot be generally recommended.

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Acknowledgements

We thank the patients and their caregiver for the cooperation. We further thank the following persons for organizational and statistical support: M. Schulz (Department of Neurology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany); M. Blicke (Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany); Ch. Ohmann, G. Felder, Q. Yang, M. Partowinia-Peters (KKS, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany); C. Schade-Brittinger (KKS Marburg).

Members of the EHDN surgical approaches working group in preparation and during the trial: (A) Rosser—Cardiff, UK (Chair); S. (B) Dunnett—Cardiff, UK (Co-Chair); J. Vesper—Düsseldorf, Germany (Co-Chair); L. Wojtecki—Düsseldorf, Germany; H. Lange—Dinslaken, Germany; (C) Saft—Bochum, Germany; R. Reilmann—Münster, Germany; S. Piacentini—Florence, Italy; A. Fasano—Rome, Italy; V. Visser-Vandewalle—Maastricht, Netherlands; Y. Temel—Maastricht, Netherlands; P. Krystowiak—Amiens, France.

Funding

The core trial was sponsored as a seed fund from the EHDN.

Author information

Authors and Affiliations

  1. Department of Neurology, Centre for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, 40225, Düsseldorf, Germany

    Stefano Ferrea, Stefan J. Groiss, Saskia Elben, Christian J. Hartmann, Alfons Schnitzler & Lars Wojtecki

  2. Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Universitaetsstrasse 1, 40225, Düsseldorf, Germany

    Stefano Ferrea, Stefan J. Groiss, Saskia Elben, Christian J. Hartmann, Alfons Schnitzler & Lars Wojtecki

  3. Brain Repair Group, School of Biosciences, Cardiff University, CF10 3AT, Cardiff, Wales, UK

    Steve B. Dunnett & Anne Rosser

  4. Department of Neurology, St. Josef-Hospital, Ruhr University, Gudrunstrasse 56, 44791, Bochum, Germany

    Carsten Saft

  5. Department of Stereotactic and Functional Neurosurgery, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, 40225, Düsseldorf, Germany

    Jan Vesper

Authors
  1. Stefano Ferrea
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  2. Stefan J. Groiss
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  3. Saskia Elben
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  4. Christian J. Hartmann
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  5. Steve B. Dunnett
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  6. Anne Rosser
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  7. Carsten Saft
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  8. Alfons Schnitzler
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  9. Jan Vesper
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  10. Lars Wojtecki
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Consortia

For the Surgical Approaches Working Group of the European Huntington’s Disease Network (EHDN)

  • A. Rosser
  • , S. B. Dunnett
  • , J. Vesper
  • , L. Wojtecki
  • , H. Lange
  • , C. Saft
  • , R. Reilmann
  • , S. Piacentini
  • , A. Fasano
  • , V. Visser-Vandewalle
  • , Y. Temel
  •  & P. Krystowiak

Corresponding author

Correspondence to Stefano Ferrea.

Ethics declarations

Ethical standards

The trial was performed in accordance with good clinical practice, fulfilled the CONSORT criteria, was registered at ClinicalTrials.gov (NCT00902889), and received approval by the local authorities according to the German Medical Devices Act (MPG) and the ethics committee of the Medical Faculty of the Heinrich-Heine University Düsseldorf (3100). Both patients gave their informed consent prior to their inclusion in the study.

Conflicts of interest

The authors declare that they have no conflict of interest.

Additional information

The members of the Surgical Approaches Working Group of the European Huntington’s Disease Network (EHDN) are listed in Acknowledgements.

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Ferrea, S., Groiss, S.J., Elben, S. et al. Pallidal deep brain stimulation in juvenile Huntington's disease: local field potential oscillations and clinical data. J Neurol 265, 1573–1579 (2018). https://doi.org/10.1007/s00415-018-8880-1

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  • DOI: https://doi.org/10.1007/s00415-018-8880-1

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