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.
Subject(s)
Dystonic Disorders/etiology , Histiocytosis, Langerhans-Cell/diagnosis , 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 useABSTRACT
OBJECTIVE: The group B streptococcal meningitis (GBS) remains an important cause of child's morbidity and mortality. The purposes of this work were to appreciate the GBS place among the bacterial meningitis, to define clinical and biological factors associated with death and to study the immediate complications. PATIENTS AND METHODS: This study concerned 276 GBS meningitis listed by the observatory of GPIP/ACTIV on the child's bacterial meningitis in France from January, 2001 to December, 2005. This report is one of the biggest series of child's GBS meningitis published to this day. RESULTS: The GBS was the third germ in frequency responsible for bacterial meningitis at every age (13 %) : 65 % of the GBS meningitis affected infants less than one month of age, 29 % infants between 1 month and 3 months of age and only 6 % infants more than 3 months of age. The male/female ratio in GBS meningitis was nearly equal (51 % vs 49 %). There was no variation of the GBS meningitis number according to the season. The serotype III was mostly involved in this cohort (81 %) followed by the serotype I (13 %). Statistically we observed a significant decline in the number of early onset meningitis from 2001 to 2004 whereas the number of late meningitis and the total meningitis cases were stable. The biological results mostly present in child's GBS meningitis were a high CSF protein level (95 %), a CSF neutrophil count more than fifteen percent (90 %), a low CSF glucose level (83.5 %), leukopenia (49 %) or a normal blood level leukocyte (38 %). Eighty three percent of the children affected by GBS meningitis were term infants. However, the prematurity seemed to be a risk factor to develop late meningitis (age between 7 days and 3 months) and very late meningitis (after 3 months) but no early onset meningitis. Prematurity, convulsions, shock, coma, assisted ventilation, high CSF protein level, weak CSF cell level and leukopenia seemed to be factors associated with death in the GBS meningitis. The mortality of the GBS meningitis was still 14 % and the immediate complications were observed in 62 % of cases, the most frequent being convulsions (45 %). CONCLUSION: The GBS meningitis in children remains a frequent problem in pediatrics despite intra partum antibioprophylaxy and thus must encourage to strengthen the prevention of these meningitis.
