Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration

Autor: Pedro J. Garcia-Ruiz, Joaquin Ayerbe, Lydia Vela Desojo, Cici E. Feliz, Javier del Val Fernandez
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Zdroj: Case Reports in Neurological Medicine, Vol 2015 (2015)
Druh dokumentu: article
ISSN: 2090-6668
2090-6676
DOI: 10.1155/2015/245735
Popis: 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.
Databáze: Directory of Open Access Journals