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Pallidal stimulation improves pantothenate kinase–associated neurodegeneration
Author(s) -
Castelnau Pierre,
Cif Laura,
Valente Enza Maria,
Vayssiere Nathalie,
Hemm Simone,
Gannau Amandine,
DiGiorgio Annalisa,
Coubes Philippe
Publication year - 2005
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.20457
Subject(s) - dystonia , neurodegeneration , globus pallidus , deep brain stimulation , medicine , stimulation , neuroscience , physical medicine and rehabilitation , psychology , basal ganglia , parkinson's disease , disease , central nervous system
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