title: Deep brain stimulation in a dentatorubral-pallidoluyisian atrophy patient with myoclonic dystonia.
abstract: We describe a patient with myoclonic dystonia caused by dentatorubral-pallidoluyisian atrophy (DRPLA), which was successfully controlled with bilateral deep brain stimulation (DBS) of the globus pallidus internus (GPi). DRPLA is a rare disease which can progressively cause a loss or degeneration of neurons in the globus pallidus, dentate nucleus, subthalamic nucleus, and red nucleus. This observation is another example of secondary dystonia which can be controlled by GPi-DBS in carefully selected patients.
authors.affiliation: Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 135-710, Republic of Korea.
authors.forename: Taek Min
authors.initials: TM
authors.lastname: Nam
authors.affiliation: Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 135-710, Republic of Korea.
authors.forename: Kyung Rae
authors.initials: KR
authors.lastname: Cho
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
authors.forename: Jinyoung
authors.initials: J
authors.lastname: Youn
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
authors.forename: Jin Whan
authors.initials: JW
authors.lastname: Cho
authors.affiliation: Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 135-710, Republic of Korea. Electronic address: jilee@skku.edu.
authors.forename: Jung-Il
authors.initials: JI
authors.lastname: Lee
mesh_terms: D000328:Adult; D046690:Deep Brain Stimulation / Q000379:methods*; D020821:Dystonic Disorders / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D020191:Myoclonic Epilepsies, Progressive / Q000150:complications / Q000628:therapy*
keywords: Deep brain stimulation; Dentatorubral-pallidoluyisian atrophy; Globus pallidus internus; Myoclonic dystonia; Secondary dystonia