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

2004; Karger Publishers; Volume: 82; Issue: 2-3 Linguagem: Inglês

10.1159/000077405

1423-0372

Goran Rakočević, Goran Rakočević, Kelly Lyons, Kelly Lyons, Steven B. Wilkinson, Steven B. Wilkinson, John Overman, John Overman, Rajesh Pahwa, Rajesh Pahwa,

Biochemical and Molecular Research

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.