Dwarfism Research Today is a free monthly online journal that collates and summarizes the latest research about Dwarfism, including details on genetics, diet, mental and motor development.

Deep brain stimulation to treat hyperkinetic symptoms of Cockayne syndrome.

Hebb MO, Gaudet P, Mendez I

Department of Surgery, Division of Neurosurgery, Dalhousie University, Halifax, Nova Scotia, Canada.

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.

Published 19 January 2006 in Mov Disord, 21(1): 112-5.
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