PMID- 21166862 OWN - NLM STAT- MEDLINE DCOM- 20110908 LR - 20220317 IS - 1651-2227 (Electronic) IS - 0803-5253 (Linking) VI - 100 IP - 7 DP - 2011 Jul TI - Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis. PG - e36-9 LID - 10.1111/j.1651-2227.2010.02125.x [doi] AB - AIM: There is currently no well-accepted therapy for central nervous system Langerhans cell histiocytosis (CNS-LCH), a neuroinflammatory disease clinically characterized by often progressive, neurological symptoms including ataxia, dysarthria, dysphagia, hypertonicity, intellectual impairment and behavioural abnormalities. We applied immunomodulative/anti-inflammatory treatment on a patient with progressive CNS-LCH disease. METHOD: Intravenous immunoglobulin (IVIG) was administered monthly for 15 years to a patient with severe, image-verified neurodegenerative CNS-LCH. RESULTS: During the IVIG treatment, the neurological deterioration initially appeared to be haltered, but over time there was still some deterioration. CONCLUSIONS: IVIG may be beneficial in partly haltering CNS-LCH neurodegeneration, but further studies are needed. CI - (c) 2011 The Author(s)/Acta Paediatrica (c) 2011 Foundation Acta Paediatrica. FAU - Gavhed, Desiree AU - Gavhed D AD - Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. FAU - Laurencikas, Evaldas AU - Laurencikas E FAU - Akefeldt, Selma O AU - Akefeldt SO FAU - Henter, Jan-Inge AU - Henter JI LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110111 PL - Norway TA - Acta Paediatr JT - Acta paediatrica (Oslo, Norway : 1992) JID - 9205968 RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunologic Factors) SB - IM MH - Adolescent MH - Central Nervous System Diseases/*drug therapy MH - Child MH - Child, Preschool MH - Disease Progression MH - Follow-Up Studies MH - Histiocytosis, Langerhans-Cell/*drug therapy MH - Humans MH - Immunoglobulins, Intravenous/*therapeutic use MH - Immunologic Factors/*therapeutic use MH - Infant MH - Male MH - Time Factors MH - Treatment Outcome EDAT- 2010/12/21 06:00 MHDA- 2011/09/09 06:00 CRDT- 2010/12/21 06:00 PHST- 2010/12/21 06:00 [entrez] PHST- 2010/12/21 06:00 [pubmed] PHST- 2011/09/09 06:00 [medline] AID - 10.1111/j.1651-2227.2010.02125.x [doi] PST - ppublish SO - Acta Paediatr. 2011 Jul;100(7):e36-9. doi: 10.1111/j.1651-2227.2010.02125.x. Epub 2011 Jan 11.