Pallidotomy for medically refractory status dystonicus in childhood. (4th April 2014)

• Record Type: Journal Article
• Authors: Marras, Carlo Efisio; Rizzi, Michele; Cantonetti, Laura; Rebessi, Erika; De Benedictis, Alessandro; Portaluri, Francesco; Randi, Franco; Savioli, Alessandra; Castelli, Enrico; Vigevano, Federico
• Is Part Of: Developmental medicine & child neurology. Volume 56:Number 7(2014:Jul.)
• Page Start: 649
• Page End: 656
• Publication Date: 2014-04-04
• Subjects: Child development -- Periodicals; Pediatric neurology -- Periodicals; 616.8
• DOI: 10.1111/dmcn.12420
• Languages: English
• ISSNs: 0012-1622

Abstract

<abstract abstract-type="main" id="dmcn12420-abs-0001"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="dmcn12420-sec-0001" sec-type="section"> <title>Aim</title> <p>Status dystonicus is a rare and potentially fatal condition of continuous and generalized muscle contraction that can complicate dystonia. As status dystonicus is usually refractory to traditional pharmacological therapy, alternative and invasive strategies have been developed, but so far there are no guidelines on status dystonicus management. Pallidotomy has shown good results in status dystonicus treatment.</p> </sec> <sec id="dmcn12420-sec-0002" sec-type="section"> <title>Method</title> <p>We report indications, surgical strategy, and outcome of bilateral pallidotomy in four pediatric patients (four males; mean age at surgery 11y 5mo) with secondary dystonia, who developed refractory status dystonicus. Pallidotomy was performed in the area corresponding to the mid portion of the globus pallidus internus.</p> </sec> <sec id="dmcn12420-sec-0003" sec-type="section"> <title>Results</title> <p>This procedure allowed patients to recover the pre‐status dystonicus condition, controlling dystonic postures and movements of trunk and limbs. Moreover oromandibular dystonia, which is resistant to conservative approaches and deep brain stimulation, was significantly reduced. No postoperative complications were registered.</p> </sec> <sec id="dmcn12420-sec-0004" sec-type="section"> <title>Interpretation</title> <p>Our study suggests pallidotomy as a feasible treatment in patients with secondary dystonia complicated by status dystonicus.</p> </sec> </abstract>