title: Deep brain stimulation for pantothenate kinase-associated neurodegeneration.
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.
authors: Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain.
authors: Pedro J
authors: PJ
authors: Garcia-Ruiz
authors: Department of Neurosurgery, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain.
authors: Joaquin
authors: J
authors: Ayerbe
authors: Department of Neurology, Hospital Fundacion Alcorcon, Calle Valdelaguna 1, Alcorcón, 2892 Madrid, Spain.
authors: Lydia
authors: L
authors: Vela Desojo
authors: Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain.
authors: Cici E
authors: CE
authors: Feliz
authors: Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain.
authors: Javier
authors: J
authors: Del Val Fernandez