title: Treatment of neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy.
abstract: BACKGROUND
  In rare cases, patients with Langerhans cell histiocytosis (LCH) develop neurodegenerative CNS disease (ND-CNS-LCH). Management of ND-CNS-LCH has not been established.
  
  
  METHODS
  We treated five pediatric patients with a combination of intravenous immunoglobulin (IVIG) and chemotherapy (steroid +/- vinblastine +/- 6-mercaptopurine +/- methotrexate). Prior to the therapy, three of the five patients had cerebellar ataxia while the remaining two had abnormal MRI findings without apparent neurological deficits. IVIG was given monthly or twice monthly at the dosage of 250-400 mg/kg/dose.
  
  
  RESULTS
  The four patients administered more than 23 doses of IVIG and chemotherapy remained in a stable condition and did not show significant progression signs in neurological deficits or brain MRI findings during the 30-month follow-up period (median; range: 19+ to 38+) following the initiation of therapy for ND-CNS-LCH.
  
  
  CONCLUSIONS
  The IVIG-containing treatment may be promising for ND-CNS-LCH; however, its effectiveness remains to be further tested in more patients as well as in a randomized trial.
authors: Division of Pediatrics, Takasago-seibu Hospital, Takasago, Japan. shinim95@mbox.kyoto-inet.or.jp
authors: Shinsaku
authors: S
authors: Imashuku
authors: Nagisa ' Amamoto '
authors: N'
authors: Okazaki
authors: Masahiko
authors: M
authors: Nakayama
authors: Naoto
authors: N
authors: Fujita
authors: Tetsuhiro
authors: T
authors: Fukuyama
authors: Kenichi
authors: K
authors: Koike
authors: Toshinori
authors: T
authors: Minato
authors: Ryoji
authors: R
authors: Kobayashi
authors: Akira
authors: A
authors: Morimoto
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D019636:Neurodegenerative Diseases / Q000188:drug therapy* / Q000473:pathology; D014747:Vinblastine / Q000008:administration & dosage
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D014747:Vinblastine; D015122:Mercaptopurine; D008727:Methotrexate