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.

1.
Peppe A, Pierantozzi M, Bassi A, Altibrandi MG, Brusa L, et al: Stimulation of the subthalamic nucleus compared with the globus pallidus internus in patients with Parkinson disease. J Neurosurg 2004;101:195–200.
2.
Deep-Brain Stimulation For Parkinson’s Disease Study Group: Deep-brain stimulation of the subthalamic nucleus of the pars interna of the globus pallidus in Parkinson’s disease. N Engl J Med 2001;345:956–963.
3.
Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Lagrange C, et al: Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 2007;6:223–229.
4.
Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Lagrange C, et al: Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 2005;352:459–467.
5.
Krauss JK, Yianni J, Loher T, Aziz TZ: Deep brain stimulation for dystonia. J Clin Neurophysiol 2004;21:18–30.
6.
Yamamoto T, Katayama Y, Kano T, Kobayashi K, Oshima H, et al: Deep brain stimulation for the treatment of parkinsonian, essential and poststroke tremor: a suitable stimulation method and changes in effective stimulation intensity. J Neurosurg 2004;101:201–209.
7.
Tsubokawa T, Katayama Y, Yamamoto T: Control of persistent hemiballismus by chronic thalamic stimulation. Report of two cases. J Neurosurg 1995;82:501–505.
8.
Moro E, Lang AE, Strafella AP, Poon YW, Arango PM, et al: Bilateral globus pallidus stimulation for Huntington’s disease. Ann Neurol 2004;56:290–294.
9.
Hebb MO, Garcia R, Gaudet P, Mendez IM: Bilateral stimulation of the globus pallidus internus to treat choreoathetosis in Huntington’s disease: technical case report. Neurosurgery 2006;58:E383.
10.
Fasano A, Mazzone P, Piano C, Quaranta D, Soleti F, et al: GPi-DBS in Huntington’s disease: results on motor function and cognition in a 72-year-old case. Mov Disord 2008;23:1289–1293.
11.
Biolsi B, Cif L, El Fertit H, Gil Robles S, Coubes P: Long-term follow-up of Huntington disease treated by bilateral deep brain stimulation of the internal globus pallidus. J Neurosurg 2008;109:130–132.
12.
Kang GA, Heath S, Rothlind J, Starr PA: Long-term follow up of pallidal deep brain stimulation in two cases of Huntington’s disease. J Neurol Neurosurg Psychiatry 2011;82:272–277.
13.
Ostrem JL, Racine CA, Glass GA, Grace JK, Volz MM, Heath SL, Starr PA: Subthalamic nucleus deep brain stimulation in primary cervical dystonia. Neurology 2011;76:870–878.
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