title: Pallidal stimulation for dystonia in pantothenate kinase-associated neurodegeneration.
abstract: Patients with generalized dystonia secondary to pantothenate kinase-associated neurodegeneration are traditionally treated palliatively with medical therapy. Therapeutic advances include stereotactic basal ganglia ablative techniques and, more recently, pallidal deep-brain stimulation. We report the course of dystonia in a teenage male. Bilateral microelectrode-guided pallidal deep-brain stimulators were placed while the patient was awake. Three parasagittal microelectrodes were inserted simultaneously. Two anterior microelectrodes were relatively quiet. The posterior electrode demonstrated a pattern of frequent bursts with high-frequency activity. The stimulator was therefore placed in the posterior location, which resulted in symptomatic improvement. Pallidal deep-brain stimulation appears to create a functional correction that may alter globus pallidus internus inhibitory output to the motor thalamus. The prominent, noisy bursting patterns observed in the globus pallidus internus suggests that high-frequency stimulation may improve signs of dystonia by normalizing thalamic discharge patterns.
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: Donald C
authors.initials: DC
authors.lastname: Shields
authors.forename: Nutan
authors.initials: N
authors.lastname: Sharma
authors.forename: John T
authors.initials: JT
authors.lastname: Gale
authors.forename: Emad N
authors.initials: EN
authors.lastname: Eskandar
mesh_terms: D000293:Adolescent; D046690:Deep Brain Stimulation / Q000379:methods*; D004421:Dystonia / Q000209:etiology / Q000473:pathology / Q000628:therapy; D005917:Globus Pallidus / Q000503:physiopathology* / Q000528:radiation effects; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D019636:Neurodegenerative Diseases / Q000150:complications / Q000201:enzymology / Q000473:pathology