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Pallidal stimulation improves pantothenate kinase–associated neurodegeneration

✍ Scribed by Pierre Castelnau; Laura Cif; Enza Maria Valente; Nathalie Vayssiere; Simone Hemm; Amandine Gannau; Annalisa DiGiorgio; Philippe Coubes

Book ID
101467471
Publisher
John Wiley and Sons
Year
2005
Tongue
English
Weight
101 KB
Volume
57
Category
Article
ISSN
0364-5134

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✦ Synopsis

Abstract

Pantothenate kinase–associated neurodegeneration (PKAN) causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patients with genetically confirmed PKAN who obtained a major and long‐lasting improvement of their painful spasms, dystonia, and functional autonomy. This study shows the benefits of pallidal DBS for the dystonia of PKAN patients. Ann Neurol 2005;57:738–741

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Long-term benefit to pallidal deep brain stimulation in a case of dystonia secondary to pantothenate kinase-associated neurodegeneration
✍ Martin Krause; Wolfgang Fogel; Volker Tronnier; Sabine Pohle; Konstanze Hörtnage 📂 Article 📅 2006 🏛 John Wiley and Sons 🌐 English ⚖ 94 KB 👁 1 views

## Abstract Pantothenate kinase‐associated neurodegeneration (PKAN) is a rare autosomal recessive disorder with onset in childhood and rapid progression. There is no causative and insufficient symptomatic drug therapy. Deep brain stimulation (DBS) of the internal pallidum (GPi) has been reported to