Deep brain stimulation to treat hyperkinetic symptoms of Cockayne syndrome.

Abstract

Cockayne syndrome manifests a spectrum of neurological dysfunction that includes medically intractable movement disorders. Deep brain stimulation has not been well studied in such rare neurodegenerative conditions. In this case, stimulation of the ventral intermediate nucleus of the thalamus was used to manage severe motor symptoms in a young man with Cockayne syndrome. There was a marked and progressive response to thalamic stimulation within weeks of surgery. These results suggest that patients with Cockayne syndrome should be considered for deep brain stimulation to treat refractory movement disorders.

• Authors: Matthew O Hebb, Paula Gaudet, Ivar Mendez
• Journal: Movement disorders : official journal of the Movement Disorder Society (Mov Disord) Vol. 21 Issue 1 Pg. 112-5 (Jan 2006) ISSN: 0885-3185 [Print] United States
• PMID: 16108029 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright (c) 2005 Movement Disorder Society.

Topics

• Adolescent
• Cockayne Syndrome (diagnosis, physiopathology, therapy)
• Deep Brain Stimulation
• Disability Evaluation
• Dominance, Cerebral (physiology)
• Electrodes, Implanted
• Follow-Up Studies
• Humans
• Hyperkinesis (diagnosis, physiopathology, therapy)
• Magnetic Resonance Imaging
• Male
• Neurologic Examination
• Tomography, X-Ray Computed
• Ventral Thalamic Nuclei (physiopathology)