Pallidal Stimulation for Dystonia in Pantothenate Kinase-associated Neurodegeneration

Donald C. Shields, Nutan Sharma, John T. Gale, Emad N. Eskandar

Research output: Contribution to journalArticle

26 Scopus citations

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 languageEnglish (US)
Pages (from-to)442-445
Number of pages4
JournalPediatric Neurology
Volume37
Issue number6
DOIs
StatePublished - Dec 1 2007
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology

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