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Case Reports in Neurological Medicine
Volume 2015, Article ID 245735, 3 pages
http://dx.doi.org/10.1155/2015/245735
Case Report

Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration

1Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain
2Department of Neurosurgery, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain
3Department of Neurology, Hospital Fundacion Alcorcon, Calle Valdelaguna 1, Alcorcón, 2892 Madrid, Spain

Received 7 November 2014; Revised 27 December 2014; Accepted 23 January 2015

Academic Editor: Federico Micheli

Copyright © 2015 Pedro J. Garcia-Ruiz et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Pantothenate kinase-associated neurodegeneration (PKAN) is usually associated with dystonia, which is typically severe and progressive over time. Pallidal stimulation (GPi DBS) has been carried out in selected cases of PKAN with drug-resistant dystonia with variable results. We report a 30-month follow-up study of a 30-year-old woman with PKAN-related dystonia treated with GPi DBS. Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty. This result has been maintained up to the present. GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.