Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy.

[adrenomyeloneuropathy]

Adrenomyeloneuropathy (AMN ) is one of several phenotypes of the adrenoleukodystrophy spectrum caused by mutations in the ABCD 1 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 .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 .IVIG may be considered as a last resort for treatment of refractory pain in AMN patients with indications of an inflammatory component .