Stefano Ferrea1,2, Stefan J Groiss3,4, Saskia Elben3,4, Christian J Hartmann3,4, Steve B Dunnett5, Anne Rosser5, Carsten Saft6, Alfons Schnitzler3,4, Jan Vesper7, Lars Wojtecki3,4. 1. Department of Neurology, Centre for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, 40225, Düsseldorf, Germany. Stefano.Ferrea@uni-duesseldorf.de. 2. Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Universitaetsstrasse 1, 40225, Düsseldorf, Germany. Stefano.Ferrea@uni-duesseldorf.de. 3. Department of Neurology, Centre for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, 40225, Düsseldorf, Germany. 4. Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Universitaetsstrasse 1, 40225, Düsseldorf, Germany. 5. Brain Repair Group, School of Biosciences, Cardiff University, CF10 3AT, Cardiff, Wales, UK. 6. Department of Neurology, St. Josef-Hospital, Ruhr University, Gudrunstrasse 56, 44791, Bochum, Germany. 7. Department of Stereotactic and Functional Neurosurgery, Medical Faculty, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, 40225, Düsseldorf, Germany.
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.
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.
Entities:
Keywords:
Intraoperative neurophysiology; Juvenile Huntington’s disease; Local field potentials; Pallidal deep brain stimulation
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