Bilateral Pallidotomy for Severe Dystonia in an 18-Month-Old Child with Glutaric Aciduria

Publisher: Karger

E-ISSN: 1423-0372|82|2-3|80-83

ISSN: 1011-6125

Source: Stereotactic and Functional Neurosurgery, Vol.82, Iss.2-3, 2004-08, pp. : 80-83

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Abstract

Glutaric aciduria type 1 is an inborn error of metabolism due to deficiency of glutaryl-CoA dehydrogenase. This disorder mainly affects children. The majority of patients develop a dystonic-dyskinetic syndrome. The dystonia is painful and can cause significant disability. This report documents an 18-month-old child, the youngest reported, who underwent pallidotomy for disabling dystonia. The surgery improved dystonic symptoms, especially pain in this child with minor complications related to the procedure. Pallidotomy is a reasonable option for children with dystonic symptoms secondary to glutaric aciduria.