This article is commented on by Pranzatelli on page 782 of this issue.
Cerebrospinal fluid B-cell expansion in longitudinally extensive transverse myelitis associated with neuromyelitis optica immunoglobulin G
Article first published online: 17 JUN 2011
© The Authors. Developmental Medicine & Child Neurology © 2011 Mac Keith Press
Developmental Medicine & Child Neurology
Volume 53, Issue 9, pages 856–860, September 2011
How to Cite
DALE, R. C., TANTSIS, E., MERHEB, V. and BRILOT, F. (2011), Cerebrospinal fluid B-cell expansion in longitudinally extensive transverse myelitis associated with neuromyelitis optica immunoglobulin G. Developmental Medicine & Child Neurology, 53: 856–860. doi: 10.1111/j.1469-8749.2011.03975.x
- Issue published online: 2 AUG 2011
- Article first published online: 17 JUN 2011
- PUBLICATION DATA Accepted for publication 28th February 2011. Published online 17th June 2011.
A first episode of central nervous system (CNS) demyelination may represent heterogeneous entities such as acute disseminated encephalomyelitis, clinically isolated syndrome, neuromyelitis optica (NMO), or multiple sclerosis. As new immune therapies become available, it is increasingly important to make an early diagnosis. Autoantibodies such as NMO immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein IgG are increasingly being employed to define subgroups of CNS demyelination or guide treatment. Similarly, cerebrospinal fluid (CSF) immunophenotyping can demonstrate B-lymphocyte subpopulation expansion, which has been used to guide therapy in other autoimmune CNS disorders. We present a report on a 15-year-old male with longitudinally extensive transverse myelitis with magnetic resonance imaging findings of oedema, cavitation, and gadolinium enhancement. NMO-IgG and aquaporin 4 IgG were positive; thus, we diagnosed a limited form of NMO. Acute CSF immunophenotyping revealed a 3.6% expansion of CD19 B-cell populations, whereas a comparison group of five children (4 males, age range 2–15y; mean age 7y) with other neurological disorders showed only a 0.51% expansion (SD 0.25%). In view of the diagnosis of a ‘limited form of neuromyelitis optica’, we therefore elected to treat him aggressively from the outset with a prolonged steroid regimen and mycophenylate mofetil. This case demonstrates a correlation between autoantibody production and CSF B lymphocyte expansion in an individual with CNS demyelination. These approaches could be used in individuals with a first episode of CNS demyelination to help delineate immunological subgroups and guide treatment.