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.
Original language | English (US) |
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Pages (from-to) | 442-445 |
Number of pages | 4 |
Journal | Pediatric Neurology |
Volume | 37 |
Issue number | 6 |
DOIs | |
State | Published - Dec 2007 |
Externally published | Yes |
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Neurology
- Developmental Neuroscience
- Clinical Neurology