Differential and better response to deep brain stimulation of chorea compared to dystonia in Huntington's disease.
Huntington's disease (HD) is an autosomal dominant and progressive neurodegenerative syndrome characterized by motor, cognitive and psychiatric manifestations. Chorea and dystonia are features that may be troublesome to some patients and may potentially prove unresponsive to pharmacological treatments. There are several reports on the results of globus pallidus internus deep brain stimulation (DBS) surgery for HD. In these published cases, DBS was utilized mainly to treat disabling chorea. We report our experience with 2 HD cases treated with DBS. The cases illustrate a differential response with a better outcome in the choreic presentation compared to the dystonic presentation. Additionally, DBS worsened gait features in both cases.
Departments of Neurology and Neurosurgery, University of Florida, Gainesville, FL 32607, USA.
Frances M
FM
Velez-Lago
Amanda
A
Thompson
Genko
G
Oyama
Angela
A
Hardwick
Justin M
JM
Sporrer
Pamela
P
Zeilman
Kelly D
KD
Foote
Dawn
D
Bowers
Herbert E
HE
Ward
Juan
J
Sanchez-Ramos
Michael S
MS
Okun
D000328:Adult; D002819:Chorea / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D046690:Deep Brain Stimulation / Q000379:methods; D004421:Dystonia / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D008297:Male; D016896:Treatment Outcome