1. [Inaugural trunk dystonia revealing Langerhans cell histiocytosis].
- Author
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Georget-Bouquinet E, Madhi F, Delacourt C, Billette T, Debray D, Reinert P, and Donadieu J
- Subjects
- Anti-Inflammatory Agents, Antineoplastic Agents, Phytogenic therapeutic use, Biopsy, Child, Child, Preschool, Diagnosis, Differential, Dystonic Disorders drug therapy, Dystonic Disorders pathology, Female, Follow-Up Studies, Histiocytosis, Langerhans-Cell drug therapy, Histiocytosis, Langerhans-Cell pathology, Humans, Hypothalamic Diseases diagnosis, Hypothalamic Diseases drug therapy, Hypothalamic Diseases pathology, Infant, Liver pathology, Liver Diseases diagnosis, Magnetic Resonance Imaging, Pituitary Diseases diagnosis, Pituitary Diseases drug therapy, Pituitary Diseases pathology, Prednisone therapeutic use, Skin pathology, Skin Diseases diagnosis, Ultrasonography, Vinblastine therapeutic use, Dystonic Disorders etiology, Histiocytosis, Langerhans-Cell diagnosis
- Abstract
Langerhans cell histiocytosis (LCH) is a multisystemic disease, which may present with neurological involvement. We report the case of a 20-month-old girl with initial liver and skin involvement. Initial symptoms were recurrent episodes of trunk dystonia, lasting approximately 2 months prior to the diagnosis of LCH. No brain MRI abnormality was demonstrated at initial work-up and over 7 years of follow-up, except for a postpituitary involvement noted after 3 years of follow-up. These episodes of dystonia subsided during the first week of specific LCH chemotherapy (vinblastine and steroid), suggesting that they may have resulted from hepatalgia related to the histiocytic infiltration of the liver.
- Published
- 2009
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