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BMC Neurology

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Open Access 01-12-2012 | Case report

Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy

Authors: Aia Elise Jønch, Else Rubæk Danielsen, Carsten Thomsen, Per Meden, Kirsten Svenstrup, Jørgen Erik Nielsen

Published in: BMC Neurology | Issue 1/2012

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.

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The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/12/108/prepub

Title: Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy
Authors: Aia Elise Jønch
Else Rubæk Danielsen
Carsten Thomsen
Per Meden
Kirsten Svenstrup
Jørgen Erik Nielsen
Publication date: 01-12-2012
Publisher: BioMed Central
Published in: BMC Neurology / Issue 1/2012
Electronic ISSN: 1471-2377
DOI: https://doi.org/10.1186/1471-2377-12-108

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy

Abstract

Background

Case presentation

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusion

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