title: Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
authors.affiliation: Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Désirée
authors.initials: D
authors.lastname: Gavhed
authors.forename: Evaldas
authors.initials: E
authors.lastname: Laurencikas
authors.forename: Selma O
authors.initials: SO
authors.lastname: Akefeldt
authors.forename: Jan-Inge
authors.initials: JI
authors.lastname: Henter
mesh_terms: D000293:Adolescent; D002493:Central Nervous System Diseases / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors