Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy

Aia E. Jønch*, Else R. Danielsen, Carsten Thomsen, Per Meden, Kirsten Svenstrup, Jørgen E. Nielsen

*Corresponding author for this work

    Research output: Contribution to journalArticleResearchpeer-review

    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.Case presentation: 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.Conclusion: IVIG may be considered as a last resort for treatment of refractory pain in AMN patients with indications of an inflammatory component.

    Original languageEnglish
    Article number108
    JournalBMC Neurology
    Volume12
    DOIs
    Publication statusPublished - 26 Sept 2012

    Keywords

    • Adrenomyeloneuropathy
    • IVIG treatment
    • Limb pain
    • Magnetic resonance spectroscopy

    Fingerprint

    Explore the research areas of 'Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy'.

    Cite this