Bilateral Globus Pallidus Interna Deep‐Brain Stimulation in a Patient With Myoclonus‐Dystonia: A Case Report. Issue 8 (25th February 2014)

• Record Type: Journal Article
• Authors: Kim, Ji Hee; Na, Young Cheol; Lee, Won Hee; Chang, Won Seok; Jung, Hyun Ho; Chang, Jin Woo
• Is Part Of: Neuromodulaton. Volume 17:Issue 8(2014)
• Page Start: 724
• Page End: 728
• Publication Date: 2014-02-25
• Subjects: Central nervous system -- Physiology -- Periodicals; Central nervous system -- Diseases -- Periodicals; 616.8
• DOI: 10.1111/ner.12162
• Languages: English
• ISSNs: 1094-7159

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="ner12162-sec-0001" sec-type="section"> <title>Objective</title> <p>Myoclonus‐dystonia is a movement disorder characterized by childhood onset of myoclonus and dystonia. We report a case of the epsilon‐sarcoglycan mutation‐negative myoclonus‐dystonia patient who underwent bilateral globus pallidus interna deep‐brain stimulation with subsequent improvement of both myoclonus and dystonia.</p> </sec> <sec id="ner12162-sec-0002" sec-type="section"> <title>Case Report</title> <p>A 37‐year‐old woman with myoclonic jerks and dystonia affecting predominantly the lower limbs was treated with chronic bilateral globus pallidus interna deep‐brain stimulation.</p> </sec> <sec id="ner12162-sec-0003" sec-type="section"> <title>Results</title> <p>The movement subscore of the Burke‐Fahns‐Marsden Dystonia Rating Scale was 38 before surgery and improved to 7 after 3 years. The disability subscore of the Burke‐Fahns‐Marsden Dystonia Rating Scale improved from 7 to 2. The Unified Myoclonus Rating Scale also decreased significantly from 93 to 39. No hardware‐ or stimulation‐related complications occurred during follow‐up.</p> </sec> <sec id="ner12162-sec-0004" sec-type="section"> <title>Conclusion</title> <p>This report suggests that patients with myoclonus‐dystonia may significantly benefit from bilateral globus pallidus interna deep‐brain stimulation. Larger studies of this patient population are needed to confirm the optimal target.</p> </sec> </abstract>