Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy

Research output: Contribution to journal › Journal article › Research › peer-review

Standard

Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy. / Jønch, Aia E; Danielsen, Else R; Thomsen, Carsten; Meden, Per; Svenstrup, Kirsten; Nielsen, Jørgen Erik.

In: B M C Neurology, Vol. 12, No. 108, 26.09.2012.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Jønch, AE, Danielsen, ER, Thomsen, C, Meden, P, Svenstrup, K & Nielsen, JE 2012, 'Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy', B M C Neurology, vol. 12, no. 108. https://doi.org/10.1186/1471-2377-12-108

APA

Jønch, A. E., Danielsen, E. R., Thomsen, C., Meden, P., Svenstrup, K., & Nielsen, J. E. (2012). Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy. B M C Neurology, 12(108). https://doi.org/10.1186/1471-2377-12-108

Vancouver

Jønch AE, Danielsen ER, Thomsen C, Meden P, Svenstrup K, Nielsen JE. Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy. B M C Neurology. 2012 Sep 26;12(108). https://doi.org/10.1186/1471-2377-12-108

Author

Jønch, Aia E ; Danielsen, Else R ; Thomsen, Carsten ; Meden, Per ; Svenstrup, Kirsten ; Nielsen, Jørgen Erik. / Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy. In: B M C Neurology. 2012 ; Vol. 12, No. 108.

Bibtex

@article{953dff7c031d45729af7e0718ccabcc5,
title = "Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy",
abstract = "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.",
author = "J{\o}nch, {Aia E} and Danielsen, {Else R} and Carsten Thomsen and Per Meden and Kirsten Svenstrup and Nielsen, {J{\o}rgen Erik}",
year = "2012",
month = sep,
day = "26",
doi = "10.1186/1471-2377-12-108",
language = "English",
volume = "12",
journal = "BMC Neurology",
issn = "1471-2377",
publisher = "BioMed Central Ltd.",
number = "108",

}

RIS

TY - JOUR

T1 - Intravenous immunoglobulin treatment in a patient with adrenomyeloneuropathy

AU - Jønch, Aia E

AU - Danielsen, Else R

AU - Thomsen, Carsten

AU - Meden, Per

AU - Svenstrup, Kirsten

AU - Nielsen, Jørgen Erik

PY - 2012/9/26

Y1 - 2012/9/26

N2 - 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.

AB - 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.

U2 - 10.1186/1471-2377-12-108

DO - 10.1186/1471-2377-12-108

M3 - Journal article

C2 - 23009600

VL - 12

JO - BMC Neurology

JF - BMC Neurology

SN - 1471-2377

IS - 108

ER -

ID: 40840957