Pantotenat Kinaz ile İlgili Nörodejenerasyon PKAN ‘a Bağlı Distonide Derin Beyin Stimülasyonu

Authors : Uğur IŞIK, Yasemin AKGÜN, Selçuk PEKER, Memet ÖZEK

Pages : 72-75

View : 29 | Download : 14

Publication Date : 2012-03-01

Article Type : Other Papers

Abstract :Pantothenate kinase-associated neurodegeneration PKAN is a rare autosomal recessive disorder with childhood onset and rapid progression. There is no effective medical therapy for this disorder. Stereotactic surgery consisting of thalomotomy and pallidotomy has been performed for medically refractory severe dystonia. Recently, deep brain stimulation DBS of globus pallidus GP has emerged as an alternative treatment in intractable dystonia. Three patients with PKAN-related dystonia, two of which demonstrated good response to bilateral globus pallidus internus GPi DBS are reported here
Keywords : Deep brain stimulation, dystonia, PKAN