title: Bilateral striatal necrosis, dystonia and multiple mitochondrial DNA deletions: case study and effect of deep brain stimulation.
abstract: Bilateral striatal necrosis (BSN) is relatively rare and has been related to a wide array of causes, including nuclear and mitochondrial DNA mutations. We report the clinical vignette of a patient with a 37 year-history of generalized dystonia secondary to BSN associated with multiple mitochondrial DNA deletions of undefined origin. Globus pallidus interna deep brain stimulation produced sustained benefit, with predominant improvements in disability.
authors.affiliation: Department of Neurological and Psychiatric Sciences, University of Bari, Bari, Italy.
authors.forename: Maria Stella
authors.initials: MS
authors.lastname: Aniello
authors.forename: Davide
authors.initials: D
authors.lastname: Martino
authors.forename: Vittoria
authors.initials: V
authors.lastname: Petruzzella
authors.forename: Roberto
authors.initials: R
authors.lastname: Eleopra
authors.forename: Michelangelo
authors.initials: M
authors.lastname: Mancuso
authors.forename: Rosa
authors.initials: R
authors.lastname: Dell'Aglio
authors.forename: Michele
authors.initials: M
authors.lastname: Cavallo
authors.forename: Gabriele
authors.initials: G
authors.lastname: Siciliano
authors.forename: Giovanni
authors.initials: G
authors.lastname: Defazio
mesh_terms: D000328:Adult; D003342:Corpus Striatum / Q000473:pathology*; D004272:DNA, Mitochondrial / Q000235:genetics*; D046690:Deep Brain Stimulation / Q000379:methods*; D018450:Disease Progression; D004421:Dystonia / Q000150:complications* / Q000628:therapy*; D007839:Functional Laterality / Q000502:physiology; D017353:Gene Deletion; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D028361:Mitochondrial Diseases / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D009336:Necrosis / Q000150:complications / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D004272:DNA, Mitochondrial