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Staged bilateral stereotactic pallidothalamotomy for life-threatening dystonia in a child with Hallervorden-Spatz disease.
- Source :
-
Movement Disorders . Jan2006, Vol. 21 Issue 1, p82-85. 4p. - Publication Year :
- 2006
-
Abstract
- Hallervorden-Spatz disease (HSD) is a rare disorder characterized by progressive motor dysfunction and dementia. Dystonia is the most prominent and disabling symptom, responding only to a modest extent to pharmacological therapy. At the moment, only a few cases have been reported to improve dystonia and even fewer to resolve status dystonicus for a longer period in children. The authors present the case of a 10-year-old boy who had progressive generalized dystonia, resulting in spontaneous femur fracture and life-threatening swallowing and respiratory disability. As a rescue solution, staged bilateral pallidothalamotomy was performed. Postoperatively, Burke-Fahn-Marsden Dystonia Rating Scale and Dystonia Disability Rating Scale improved (from 116 and 30 points to 41 and 18 points, respectively) and painful dystonia was resolved, which was still continuous 4 years later (47 and 20 points). Stereotactic staged bilateral pallidothalamotomy should be considered as a potential treatment in the management of life-threatening generalized dystonia related to HSD. © 2005 Movement Disorder Society [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 08853185
- Volume :
- 21
- Issue :
- 1
- Database :
- Academic Search Index
- Journal :
- Movement Disorders
- Publication Type :
- Academic Journal
- Accession number :
- 64241895
- Full Text :
- https://doi.org/10.1002/mds.20655