Deep brain stimulation to treat hyperkinetic symptoms of Cockayne syndrome.
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.
Hebb
Matthew O
MO
Department of Surgery, Division of Neurosurgery, Dalhousie University, Halifax, Nova Scotia, Canada.
Gaudet
Paula
P
Mendez
Ivar
I
D000293:Adolescent; D003057:Cockayne Syndrome / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D046690:Deep Brain Stimulation; D004185:Disability Evaluation; D004292:Dominance, Cerebral / Q000502:physiology; D004567:Electrodes, Implanted; D005500:Follow-Up Studies; D006801:Humans; D006948:Hyperkinesis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D014057:Tomography, X-Ray Computed; D020651:Ventral Thalamic Nuclei / Q000503:physiopathology