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Long‐term benefit to pallidal deep brain stimulation in a case of dystonia secondary to pantothenate kinase‐associated neurodegeneration
Author(s) -
Krause Martin,
Fogel Wolfgang,
Tronnier Volker,
Pohle Sabine,
Hörtnagel Konstanze,
Thyen Ute,
Volkmann Jens
Publication year - 2006
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21166
Subject(s) - deep brain stimulation , dystonia , neurodegeneration , medicine , neuroscience , neuroimaging , pediatrics , psychology , disease , parkinson's disease
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 improve motor function. Most case reports, however, are limited to short observational periods. The impact of DBS on the progression and life expectancy in PKAN is unknown. We present a 5‐year outcome and video documentation of bilateral GPi‐DBS of an adolescent patient suffering from genetically defined PKAN. © 2006 Movement Disorder Society