J Mov Disord.  2022 Jan;15(1):62-65. 10.14802/jmd.21005.

Deep Brain Stimulation for Cockayne Syndrome-Associated Movement Disorder

Affiliations
  • 1Department of Neurosurgery, University Kansas Medical Center, Kansas City, KS, USA
  • 2Division of Neurology, Children’s Mercy Kansas City, Kansas City, MO, USA
  • 3Division of Neurosurgery, Children’s Mercy Kansas City, Kansas City, MO, USA

Abstract

Cockayne syndrome (CS) is a rare progeroid disorder characterized by multisystem degeneration, including neurological dysfunction, for which deep brain stimulation (DBS) is a proposed treatment. This study represents only the third case of DBS for CS-associated movement disorder and the first in which both proposed targets had devices implanted, allowing for direct comparison. A case of DBS for CS-associated movement disorder is presented. Previous literature documents two cases with one targeting the ventral intermediate nucleus of the thalamus (VIM) and the other targeting the globus pallidus interna (GPi). Our patient underwent stimulation of GPi nuclei followed by repositioning to VIM nuclei with improved symptom control using VIM stimulation. In all cases, there was a significant clinical benefit without off-target effects. CS-associated movement disorder exhibits phenotypic variability for which DBS is a viable treatment. Target selection should be driven by clinical phenotype.

Keyword

Cockayne syndrome; Deep brain stimulation; Dystonia; Tremor
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