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Your search keyword '"Boetzel, K."' showing total 2 results
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2 results on '"Boetzel, K."'

1. Metaanalysis of chronic pallidal stimulation in children with DYT-1 dystonia

Author

Florian Heinen, Jan Hinnerk Mehrkens, Boetzel K, and Ingo Borggräfe

Subjects
Dystonia, Pediatrics, medicine.medical_specialty, business.industry, General Medicine, Disease, medicine.disease, Penetrance, Dopamine, Pallidal stimulation, Pediatrics, Perinatology and Child Health, Medicine, Neurology (clinical), business, Medical therapy, Severely disabling, medicine.drug
Abstract

Introduction: DYT1-dystonia is an autosomal-dominant disease with a penetrance rate of 3040% and usually starts in childhood between the age 8-12 severely disabling some patients over time. Medical therapy with anti-cholinergic drugs or dopamine is often ineffective and may produce severe side effects. Recent reports suggest that chronic bilateral pallidal stimulation leads to major improvement in these patients.

Published
2008

2. Return to Participation — Significant Improvement after Bilateral Pallidal Stimulation in Rapidly Progressive DYT-1 Dystonia.

Author

Borggraefe, I., Boetzel, K., Boehmer, J., Berweck, S., Mueller-Felber, W., MueIIer, K., Mehrkens, J. H., and Heinen, F.

Subjects
*DYSTONIA, *BRAIN stimulation, *DOPAMINE, *PARASYMPATHOLYTIC agents, *GLOBUS pallidus, *JOINT dislocations
Abstract

We report the case of an 8-year-old girl who developed progressive generalized dystonia, rendering her unable to walk and sit within months despite medical therapy with dopamine and anti-cholinergic agents. She was found to have a 9q34.1 GAG-deletion, which is known to cause DYTI-dystonia. DYT-1 dystonia is an autosomal dominant condition with incomplete penetrance that usually starts in childhood. It is known to be refractory to pharmacotherapy. Reports on deep brain stimulation in this condition reveal marked benefits of the treatment in the pediatric and adult populations. The patient underwent bilateral stimulation of the internal globus pallidus 18 months after symptom onset. Postoperatively, her clinical status improved significantly as measured by the Burke-Fahn- Marsden dystonia rating scale and the resolution of a unilateral hip dislocation. Normal participation was regained. [ABSTRACT FROM AUTHOR]

Published
2008
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