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.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Ferrea S,Groiss SJ,Elben S,Hartmann CJ,Dunnett SB,Rosser A,Saft C,Schnitzler A,Vesper J,Wojtecki L,Surgical Approaches Working Group of the European Huntington’s Disease Network (EHDN).doi
10.1007/s00415-018-8880-1subject
Has Abstractpub_date
2018-07-01 00:00:00pages
1573-1579issue
7eissn
0340-5354issn
1432-1459pii
10.1007/s00415-018-8880-1journal_volume
265pub_type
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