J Korean Neurosurg Soc.  2005 Nov;38(5):380-383.

Bilateral Pallidotomy for Dystonia with Glutaric Aciduria Type 1

• Hwang HS
• Salles AD

Abstract

Glutaric aciduria type 1 is an inborn error of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-coenzyme A dehydrogenase. The disease often appears in infancy with encephalopathy episode that results in acute basal ganglia and white matter degeneration. The majority of patients develop a dystonic-dyskinetic syndrome. This reports 6year-old boy who had been done previous gastrostomy due to swallowing difficulty underwent bilateral pallidotomy with intraoperative electromyography(EMG) monitoring for disabling dystonia. Intraoperative EMG was used to assess stimulation thresholds required for capsular responses and muscle tone. Surface EMG electrodes were placed on the face and cricopharyngeal muscles. Exact target were directly modified according to MRI-visualized anatomy. EMG response was consistently seen prior to visual observation of muscle activity. The surgery improved dystonic symptoms without swallowing difficulty.

Keyword

Glutaric aciduria; Dystonia; Pallidotomy

MeSH Terms

Basal Ganglia
Deglutition
Dystonia*
Electrodes
Gastrostomy
Glutaryl-CoA Dehydrogenase
Humans
Hydroxylysine
Lysine
Male
Metabolism
Muscles
Pallidotomy*
Tryptophan
Glutaryl-CoA Dehydrogenase
Hydroxylysine
Lysine
Tryptophan