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Ann Neurol. 2005 May;57(5):738-41.

Pallidal stimulation improves pantothenate kinase-associated neurodegeneration.

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1
Pediatric Neurology and Institut National de la Sante et de la Recherche Médicale UB19 Hôpital Gatien de Clocheville, Centre Hospitalo-Universitaire, Tours, France 619.

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.

PMID:
15852393
DOI:
10.1002/ana.20457
[Indexed for MEDLINE]
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