Wilson's disease (WD) is an autosomal recessive disorder of copper metabolism associated with ATP7B gene mutations. In this condition, copper accumulates in various tissues especially the brain, and liver. The resulting cellular dysfunction commonly manifests as ataxia, tremor, dystonia and parkinsonism. Whilst copper chelation therapies, including trientene and penicillamine, are well-established disease-modifying treatments, up to 50% of patients have persistent neurological symptoms despite optimal non-surgical therapy [1]. Brain lesioning [2] and deep brain stimulation (DBS) [3,4] have been occasionally used but this is not established treatment and there is no consensus on the optimal target. Reported surgical targets include the Vim/Voa thalamic nuclei for tremor [2,3] and globus pallidus interna (GPi) for hemiballismus and axial dystonia [4]. Although the subthalamic nucleus (STN) has been targeted for dystonia control [5,6] we could not, at the time of writing, find any published articles on its use in WD. Indeed, there is an ongoing trial seeking to determine whether the STN or the GPi is a better target for stimulation in patients with WD [7].

1. Hölscher, S., Leinweber, B., Hefter, H., et al. (2010). Evaluation of the symptomatic treatment of residual neurological symptoms in Wilson disease. European Neurology, 64(2), 83–87. https://doi.org/10.1159/000315140

2. Pal, P. L., Sinha, S., Taly, A. B., et al. (2007). Successful treatment of tremor in Wilson’s disease by thalamotomy: A case report. Movement Disorders, 22(15), 2287–2290. https://doi.org/10.1002/mds.21665

3. Hedera, P. (2014). Treatment of Wilson’s disease motor complications with deep brain stimulation. Annals of the New York Academy of Sciences, 1315(1), 16–23. https://doi.org/10.1111/nyas.12412

4. Sidiropoulos, C., Hutchison, W., Mestre, T., et al. (2013). Bilateral pallidal stimulation for Wilson’s disease. Movement Disorders, 28(9), 1292–1294. https://doi.org/10.1002/mds.25493

5. Ostrem, J. L., Luciano, M. S., Dodenhoff, K. A., et al. (2016). Subthalamic nucleus deep brain stimulation in isolated dystonia: A 3-year follow-up study. Neurology, 88(1), 25–35. https://doi.org/10.1212/WNL.0000000000003451

6. Fonoff, E. T., Campos, W. K., Mandel, M., et al. (2012). Bilateral subthalamic nucleus stimulation for generalized dystonia after bilateral pallidotomy. Movement Disorders, 27(12), 1559–1563. https://doi.org/10.1002/mds.25193

7. WILSTIM-DBS (WILsonSTIMulation–Deep Brain Stimulation). (n.d.). ClinicalTrials.gov. Retrieved December 9, 2018, from https://clinicaltrials.gov/ct2/show/NCT02552628

8. Plaha, P., Khan, S., & Gill, S. S. (2008). Bilateral stimulation of the cauda zona incerta nucleus for tremor control. Journal of Neurology, Neurosurgery & Psychiatry, 79(5), 504–513. https://doi.org/10.1136/jnnp.2006.112334

9. Velasco, F., Jiménez, F., Pérez, M. L., et al. (2001). Electrical stimulation of the prelemniscal radiation in the treatment of Parkinson’s disease: An old target revised with new techniques. Neurosurgery, 49(2), 293–306. https://doi.org/10.1097/00006123-200108000-00008

10. Ito, Z. (1975). Stimulation and destruction of the prelemniscal radiation or its adjacent area in various extrapyramidal disorders. Confinia Neurologica, 37(1–3), 41–48. https://doi.org/10.1159/000102365