Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington's disease: technical case report.
OBJECTIVE
Huntington's disease (HD) produces debilitating motor abnormalities that are poorly responsive to medical therapy. Deep brain stimulation (DBS) may offer a treatment option for afflicted patients, but its role in the management of HD remains unclear. In the present case, DBS leads were implanted bilaterally into the posteroventral globus pallidus internus (GPi) to control disabling and medically intractable choreathetosis in a severely affected HD patient. The surgical procedure, intraoperative electrophysiological findings, and 12-month postoperative course, with patient video, are presented.


METHODS
This 41-year-old man with genetically confirmed HD developed motor symptoms at age 28. He had completed multiple medical trials without alleviation of his progressive and debilitating choreathetosis. Extensive clinical assessment, including neuropsychological testing, was performed to determine surgical candidacy.


METHODS
DBS leads were bilaterally implanted, under stereotactic guidance, into the posteroventral GPi. Disease progression and symptom control were assessed at regular postoperative intervals. Bilateral pallidal stimulation produced a dramatic reduction in choreathetoid movements and improvement in overall motor functioning. The patient also exhibited normalization of body weight, mood, and energy level, as well as improved performance of activities of daily living. These effects were sustained at 1 year after surgery.


CONCLUSIONS
The clinical benefits of DBS observed in this HD patient were comparable to those reported in other hyperkinetic disorders and demonstrate that pallidal stimulation can provide long-term alleviation of HD-associated choreathetosis.
Hebb
Matthew O
MO
Division of Neurosurgery, Department of Surgery, Dalhousie University, Halifax, Nova Scotia, Canada.
Garcia
Rene
R
Gaudet
Paula
P
Mendez
Ivar M
IM
D000328:Adult; D001264:Athetosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D002819:Chorea / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D046690:Deep Brain Stimulation / Q000379:methods*; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D016343:Monitoring, Intraoperative / Q000379:methods