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. 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.

journal_name

BMC Neurol

journal_title

BMC neurology

authors

Jønch AE,Danielsen ER,Thomsen C,Meden P,Svenstrup K,Nielsen JE

doi

10.1186/1471-2377-12-108

subject

Has Abstract

pub_date

2012-09-26 00:00:00

pages

108

issn

1471-2377

pii

1471-2377-12-108

journal_volume

12

pub_type

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