Document Detail


Bilateral subthalamic nucleus deep brain stimulation for dopa-responsive dystonia in a 6-year-old child.
MedLine Citation:
PMID:  21631204     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Tyrosine hydroxylase (TH) deficiency is a rare autosomal recessive metabolic disease that results in the decreased production of catecholamines. Standard treatment relies on combinations of levodopa and carbidopa, anticholinergic agents, serotonergic agonists, and monamine oxidase B inhibitors. Unfortunately, severely affected children often require escalating doses of medication and suffer from dyskinesias as well as significant on/off symptomatology. The authors present a case of medically intractable dopa-responsive dystonia in a 6-year-old boy whose condition significantly improved with bilateral subthalamic nucleus deep brain stimulation. This case is unique in its novel approach to tyrosine hydroxylase deficiency and the young age of the patient.
Authors:
Matthew J Tormenti; Nestor D Tomycz; Keith A Coffman; Douglas Kondziolka; Donald J Crammond; Elizabeth C Tyler-Kabara
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of neurosurgery. Pediatrics     Volume:  7     ISSN:  1933-0715     ISO Abbreviation:  J Neurosurg Pediatr     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-06-02     Completed Date:  2011-08-19     Revised Date:  2011-12-13    
Medline Journal Info:
Nlm Unique ID:  101463759     Medline TA:  J Neurosurg Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  650-3     Citation Subset:  IM    
Affiliation:
UPMC Center for Brain Function and Behavior, Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
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MeSH Terms
Descriptor/Qualifier:
Child
Deep Brain Stimulation* / methods
Dyskinesias / congenital,  therapy*
Dystonic Disorders / congenital,  physiopathology,  therapy
Humans
Male
Subthalamic Nucleus / physiopathology*
Treatment Outcome
Comments/Corrections
Erratum In:
J Neurosurg Pediatr. 2011 Nov;8(5):536
Note: Crammond, Donald J [added]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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