Pallidal stimulation improves pantothenate kinase-associated neurodegeneration.
case_series · Level IV
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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.
Medical subject headings
- Deep Brain Stimulation
- Globus Pallidus
- Neurodegenerative Diseases
- Phosphotransferases (Alcohol Group Acceptor)