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Successful bilateral pallidal stimulation in a patient with isolated lower limb dystonia coexistent with Langerhans cell histiocytosis and coeliac disease
Affiliations- Klinika Neurochirurgii i Urazów Ośrodkowego Układu Nerwowego, Centrum Medyczne Kształcenia Podyplomowego w Warszawie
- Katedra i Kliniczny Oddział Neurochirurgii w Sosnowcu, Śląski Uniwersytet Medyczny w Katowicach
- Poradnia Genetyczna, Zakład Genetyki Medycznej, Instytut Matki i Dziecka w Warszawie
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Abstract
The authors report a case of bilateral globus pallidus internus (GPi) stimulation for treatment of medically intractable isolated lower limb dystonia. The 14-year-old girl developed dystonic movements in her left lower limb. At the age of 17, the patient was handicapped by dystonic movements in her lower limbs, and became wheelchair-bound. Pharmacological therapy and botulinum toxin injection resulted in transient and modest benefit. Moreover, the patient was diagnosed with histologically proven coeliac disease and Langerhans cell histiocytosis. Genetic testing revealed the presence of DYT-1 mutation. The 17-year-old girl underwent bilateral implantation of deep brain stimulation leads. Bilateral GPi stimulation resulted in remarkable improvement of phasic dystonic movements, and dystonic posture of lower limbs. Over 2 years postoperative follow-up, the patient is able to walk independently. Bilateral GPi stimulation appears to be an effective treatment modality for isolated lower limb dystonia.
Abstract
The authors report a case of bilateral globus pallidus internus (GPi) stimulation for treatment of medically intractable isolated lower limb dystonia. The 14-year-old girl developed dystonic movements in her left lower limb. At the age of 17, the patient was handicapped by dystonic movements in her lower limbs, and became wheelchair-bound. Pharmacological therapy and botulinum toxin injection resulted in transient and modest benefit. Moreover, the patient was diagnosed with histologically proven coeliac disease and Langerhans cell histiocytosis. Genetic testing revealed the presence of DYT-1 mutation. The 17-year-old girl underwent bilateral implantation of deep brain stimulation leads. Bilateral GPi stimulation resulted in remarkable improvement of phasic dystonic movements, and dystonic posture of lower limbs. Over 2 years postoperative follow-up, the patient is able to walk independently. Bilateral GPi stimulation appears to be an effective treatment modality for isolated lower limb dystonia.
Keywords
movement disorders, lower limb dystonia, pallidal stimulation, functional neurosurgery
About this articleTitle
Successful bilateral pallidal stimulation in a patient with isolated lower limb dystonia coexistent with Langerhans cell histiocytosis and coeliac disease
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
514-519
Page views
193
Article views/downloads
358
DOI
10.1016/S0028-3843(14)60322-4
Bibliographic record
Neurol Neurochir Pol 2011;45(5):514-519.
Keywords
movement disorders
lower limb dystonia
pallidal stimulation
functional neurosurgery
Authors
Michał Sobstyl
Mirosław Ząbek
Sebastian Dzierzęcki
Zbigniew Mossakowski
Krzysztof Szczałuba
