Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration
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 fol...
Saved in:
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2015-01-01
|
| Series: | Case Reports in Neurological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/245735 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832562931160580096 |
|---|---|
| author | Pedro J. Garcia-Ruiz Joaquin Ayerbe Lydia Vela Desojo Cici E. Feliz Javier del Val Fernandez |
| author_facet | Pedro J. Garcia-Ruiz Joaquin Ayerbe Lydia Vela Desojo Cici E. Feliz Javier del Val Fernandez |
| author_sort | Pedro J. Garcia-Ruiz |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-264bee4338364beaa1e2dde61a9a181f |
| institution | Kabale University |
| issn | 2090-6668 2090-6676 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Neurological Medicine |
| spelling | doaj-art-264bee4338364beaa1e2dde61a9a181f2025-02-03T01:21:24ZengWileyCase Reports in Neurological Medicine2090-66682090-66762015-01-01201510.1155/2015/245735245735Deep Brain Stimulation for Pantothenate Kinase-Associated NeurodegenerationPedro J. Garcia-Ruiz0Joaquin Ayerbe1Lydia Vela Desojo2Cici E. Feliz3Javier del Val Fernandez4Department of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, SpainDepartment of Neurosurgery, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, SpainDepartment of Neurology, Hospital Fundacion Alcorcon, Calle Valdelaguna 1, Alcorcón, 2892 Madrid, SpainDepartment of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, SpainDepartment of Neurology, Fundacion Jimenez Diaz, Avenida Reyes Catolicos 2, 28040 Madrid, SpainPantothenate 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.http://dx.doi.org/10.1155/2015/245735 |
| spellingShingle | Pedro J. Garcia-Ruiz Joaquin Ayerbe Lydia Vela Desojo Cici E. Feliz Javier del Val Fernandez Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration Case Reports in Neurological Medicine |
| title | Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration |
| title_full | Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration |
| title_fullStr | Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration |
| title_full_unstemmed | Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration |
| title_short | Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration |
| title_sort | deep brain stimulation for pantothenate kinase associated neurodegeneration |
| url | http://dx.doi.org/10.1155/2015/245735 |
| work_keys_str_mv | AT pedrojgarciaruiz deepbrainstimulationforpantothenatekinaseassociatedneurodegeneration AT joaquinayerbe deepbrainstimulationforpantothenatekinaseassociatedneurodegeneration AT lydiaveladesojo deepbrainstimulationforpantothenatekinaseassociatedneurodegeneration AT ciciefeliz deepbrainstimulationforpantothenatekinaseassociatedneurodegeneration AT javierdelvalfernandez deepbrainstimulationforpantothenatekinaseassociatedneurodegeneration |