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Bilateral striatal necrosis, dystonia and multiple mitochondrial DNA deletions: Case study and effect of deep brain stimulation

✍ Scribed by Maria Stella Aniello; Davide Martino; Vittoria Petruzzella; Roberto Eleopra; Michelangelo Mancuso; Rosa Dell'Aglio; Michele Cavallo; Gabriele Siciliano; Giovanni Defazio


Publisher
John Wiley and Sons
Year
2008
Tongue
English
Weight
144 KB
Volume
23
Category
Article
ISSN
0885-3185

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✦ Synopsis


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 years‐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. © 2007 Movement Disorder Society