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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. |
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basal ganglia,lesion of
dystonia
dystonia musculorum deformens
dystonia,symptomatic
dystonia,treatment of
gene mutation
globus pallidus,stimulation
iron,brain
MRI,abnormal
MRI,eye of tiger sign
MRI,paramagnetic effect
PANK2 mutation
stimulation,deep brain
symmetric brain lesions
treatment of neurologic disorder
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