Pallidal Stimulation in Siblings with Pantothenate Kinase-Associated Neurodegeneration: Four-Year Follow-Up
2011
Abstract
Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive neurodegenerative brain disorder manifested by progressive motor and cognitive impairment, often with a fatal outcome in early adulthood. Complications may involve painful generalized dystonia interfering with food intake or breathing, and may lead to malnutrition and complete immobility of the patient.
Since 2002, deep brain stimulation (DBS) of globus pallidus interna (GPi) has been successfully used in PKAN patients; nevertheless, little is known about its long-term outcome.