ISSN 1738-6217 (Print)
 
 
 
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Abstract

J Kor Soc Ster Func Neurosurg. 2018 14(2): 47-50
ⓒ Journal of the Korean Society of Stereotactic and Functional Neurosurgery
    
 
Globus Pallidus Internus Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration Dystonia
Min Seung Myung, MD1, Jinyoung Youn, MD, PhD2, Jung-Il Lee, MD, PhD1, Kyung Rae Cho, MD1
1Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul, Korea 2Department of Neurology, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul, Korea
 

Pantothenate kinase-associated neurodegeneration (PKAN) represents an autosomal recessive hereditary disease with progressive pharmacologically intractable dystonia. In this report, after consultation to neurologist for dystonia, a PANK2 gene mutation in a 50 year-old woman was identified. She suffered from dystonia and dystonic pain which cause difficulty in activity of daily living. We performed bilateral internal globus pallidus stimulation (GPi DBS) and the benefit was evident. She showed marked improvement in dystonia and dystonic pain with a significant reduction in disability. This study shows the importance of cooperation with neurologist and the benefits of GPi DBS for the dystonia of PKAN patient. To conclude, bilateral pallidal stimulation is an effective and safe treatment for intractable dystonia in PKAN.
 
Key words : Deep brain stimulation, PKAN, PANK2, Dystonia.
중심단어 : Deep brain stimulation, PKAN, PANK2, Dystonia.
 
 
  
 
 
 

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