Staged bilateral stereotactic pallidothalamotomy for life-threatening dystonia in a child with Hallervorden-Spatz disease

Istvan Balas, N. Kovács, Katalin Hollody

Research output: Contribution to journalArticle

44 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)82-85
Number of pages4
JournalMovement Disorders
Volume21
Issue number1
DOIs
Publication statusPublished - Jan 2006

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Pantothenate Kinase-Associated Neurodegeneration
Dystonia
Spontaneous Fractures
Deglutition
Femur
Dementia
Pharmacology

Keywords

  • Dystonia
  • Hallervorden
  • Pallidotomy
  • Spatz disease
  • Thalamotomy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Staged bilateral stereotactic pallidothalamotomy for life-threatening dystonia in a child with Hallervorden-Spatz disease. / Balas, Istvan; Kovács, N.; Hollody, Katalin.

In: Movement Disorders, Vol. 21, No. 1, 01.2006, p. 82-85.

Research output: Contribution to journalArticle

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