title: Successful bilateral pallidal stimulation in a patient with isolated lower limb dystonia coexistent with Langerhans cell histiocytosis and coeliac disease.
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.
authors: Klinika Neurochirurgii, Centrum Medycznego Kształcenia Podyplomowego, ul. Marymoncka 99, 01-813 Warszawa. mrsob@op.pl
authors: Michał
authors: M
authors: Sobstyl
authors: Mirosław
authors: M
authors: Ząbek
authors: Sebastian
authors: S
authors: Dzierzęcki
authors: Zbigniew
authors: Z
authors: Mossakowski
authors: Krzysztof
authors: K
authors: Szczałuba
mesh_terms: D000293:Adolescent; D002446:Celiac Disease / Q000150:complications / Q000628:therapy*; D046690:Deep Brain Stimulation / Q000295:instrumentation*; D004421:Dystonia / Q000150:complications / Q000628:therapy*; D004567:Electrodes, Implanted; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000503:physiopathology*; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000628:therapy*; D006801:Humans; D019635:Neurosurgical Procedures / Q000379:methods; D016896:Treatment Outcome