title: Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy.
abstract: BACKGROUND
  Adrenomyeloneuropathy (AMN) is one of several phenotypes of the adrenoleukodystrophy spectrum caused by mutations in the ABCD1 gene on the X chromosome. An inflammatory component is part of the disease complex ranging from severe childhood CNS demyelination to spinal cord and peripheral nerve degeneration.
  
  
  METHODS
  We present a patient with clinical progressive AMN and severe lower limb pain. Longitudinal brain magnetic resonance spectroscopy showed a constant slightly elevated myoinositol/total creatine ratio during the five year treatment period, probably reflecting demyelination, microglial activation and gliosis, indicating an inflammatory response. The pain was refractory to conventional therapy but intravenous immunoglobulin (IVIG) treatment was highly efficient.
  
  
  CONCLUSIONS
  IVIG may be considered as a last resort for treatment of refractory pain in AMN patients with indications of an inflammatory component.
authors.affiliation: Kennedy Centre, Copenhagen University Hospital, Rigshospitalet, Gl, Landevej 7, Copenhagen, 2600, Glostrup, Denmark. aia@kennedy.dk
authors.forename: Aia Elise
authors.initials: AE
authors.lastname: Jønch
authors.forename: Else Rubæk
authors.initials: ER
authors.lastname: Danielsen
authors.forename: Carsten
authors.initials: C
authors.lastname: Thomsen
authors.forename: Per
authors.initials: P
authors.lastname: Meden
authors.forename: Kirsten
authors.initials: K
authors.lastname: Svenstrup
authors.forename: Jørgen Erik
authors.initials: JE
authors.lastname: Nielsen
mesh_terms: D000326:Adrenoleukodystrophy / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents