[Inaugural trunk dystonia revealing Langerhans cell histiocytosis].
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.
Service de pédiatrie générale, hôpital intercommunal de Créteil, 40, avenue de Verdun, 94000 Créteil, France. emilie.georget@chicreteil.fr
E
E
Georget-Bouquinet
F
F
Madhi
C
C
Delacourt
T
T
Billette
D
D
Debray
P
P
Reinert
J
J
Donadieu
D000893:Anti-Inflammatory Agents; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D020821:Dystonic Disorders / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007027:Hypothalamic Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D007223:Infant; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D010900:Pituitary Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000175:diagnosis; D014463:Ultrasonography; D014747:Vinblastine / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D000972:Antineoplastic Agents, Phytogenic; D014747:Vinblastine; D011241:Prednisone