Pallidal stimulation in siblings with pantothenate kinase-associated neurodegeneration: Four-year follow-up
Abstract
PKAN - pantothenate kinase-associated neurodegeneration, formerly Hallervorden-Spatz disease is a devastating disease. One of the main symptoms is pharmacoresistant generalised dystonia.
Neurosurgical treatmen of generalised dystonia - deep brain stimulation of pallidum internum (DBS GPi) has been successfully used in PKAN patients since 2002. We report 2 siblings with PKAN treated by DBS GPi.
Clinically dominated pharmacoresistant generalised dystonia - axial, oromandibular and in extremities. In the boy, Burke-Fahn-Marsden dystonia rating scale improved by 81% respectively 51% 3 months respectively 4 years and 5 months after operation.
In the girl, the improvement was 41% respectively 28% 3 months respectively 4 years after operation. There were no side effects and both patients benefited from the treatment.