Stereotactic pallidotomy in a child with Hallervorden-Spatz disease. Case report
- PMID: 10067928
- DOI: 10.3171/jns.1999.90.3.0551
Stereotactic pallidotomy in a child with Hallervorden-Spatz disease. Case report
Abstract
The authors present a case of Hallervorden-Spatz disease (HSD) in a 10-year-old boy treated with stereotactic pallidotomy for control of severe dystonia. Hallervorden-Spatz disease is a rare type of neuraxonal dystrophy that can be familial or sporadic. This is the first case of HSD reported in the literature in which a pallidotomy was performed. The patient had progressively worsening dystonias and spasms that prevented useful function of his entire right side and eventually threatened his respiratory ability. Pre- and postoperative magnetic resonance images are presented along with electrophysiological recordings made in the globus pallidus at the time of surgery. Functional improvement in the use of the patient's limbs and relief from the painful dystonia were observed. Stereotactic pallidotomy should be considered as a potential treatment in the management of HSD.
Similar articles
-
Staged bilateral stereotactic pallidothalamotomy for life-threatening dystonia in a child with Hallervorden-Spatz disease.Mov Disord. 2006 Jan;21(1):82-5. doi: 10.1002/mds.20655. Mov Disord. 2006. PMID: 16108022
-
Status dystonicus and Hallervorden-Spatz disease: treatment with intrathecal baclofen and pallidotomy.J Paediatr Child Health. 2004 May-Jun;40(5-6):322-5. doi: 10.1111/j.1440-1754.2004.00374.x. J Paediatr Child Health. 2004. PMID: 15151592
-
Pallidal deep brain stimulation for longstanding severe generalized dystonia in Hallervorden-Spatz syndrome. Case report.J Neurosurg. 2004 Apr;100(4):706-9. doi: 10.3171/jns.2004.100.4.0706. J Neurosurg. 2004. PMID: 15070127
-
Follow-up magnetic resonance imaging in Hallervorden-Spatz disease.Clin Neurol Neurosurg. 1992;94 Suppl:S57-60. doi: 10.1016/0303-8467(92)90023-v. Clin Neurol Neurosurg. 1992. PMID: 1320520 Review.
-
Hallervorden spatz disease: MR and pathological findings of a rare case.Neurol India. 2005 Mar;53(1):102-4. Neurol India. 2005. PMID: 15805666 Review.
Cited by
-
Pantothenate kinase-associated neurodegeneration: Clinical aspects, diagnosis and treatments.Neurol Int. 2018 Apr 4;10(1):7516. doi: 10.4081/ni.2018.7516. eCollection 2018 Mar 30. Neurol Int. 2018. PMID: 29844889 Free PMC article.
-
Genetics and Pathophysiology of Neurodegeneration with Brain Iron Accumulation (NBIA).Curr Neuropharmacol. 2013 Jan;11(1):59-79. doi: 10.2174/157015913804999469. Curr Neuropharmacol. 2013. PMID: 23814539 Free PMC article.
-
What happened to posteroventral pallidotomy for Parkinson's disease and dystonia?Neurotherapeutics. 2008 Apr;5(2):281-93. doi: 10.1016/j.nurt.2008.02.001. Neurotherapeutics. 2008. PMID: 18394570 Free PMC article. Review.
-
Towards Precision Therapies for Inherited Disorders of Neurodegeneration with Brain Iron Accumulation.Tremor Other Hyperkinet Mov (N Y). 2021 Nov 24;11:51. doi: 10.5334/tohm.661. eCollection 2021. Tremor Other Hyperkinet Mov (N Y). 2021. PMID: 34909266 Free PMC article. Review.
-
Anaesthetic management of a child with panthothenate kinase-associated neurodegeneration.Indian J Anaesth. 2015 Jan;59(1):43-6. doi: 10.4103/0019-5049.149449. Indian J Anaesth. 2015. PMID: 25684813 Free PMC article.
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
