The Turkish Journal of Pediatrics
2014 , Vol 56 , Num 6
Neuromyelitis Optica in Children: A Review of the Literature
1Department of Neurology, Baskent University Faculty of Medicine, 2Department of Neurology, Ankara Training and
Research Hospital, 3Division of Pediatric Neurology, Department of Pediatrics, and 4Department of Neurology, Hacettepe
University Faculty of Medicine, Ankara, Turkey. E-mail: edaderle@hotmail.com
Neuromyelitis optica (NMO) is a rare and severe inflammatory disease of
the central nervous system (CNS), which constitutes up to 5% of pediatric
aquired demyelinating diseases. The optic nerves and the spinal cord are the
most affected sites. The discovery of an autoantibody called NMO-IgG, which
targets aquaporin-4, the main water channel in the CNS, gave a new direction
to understanding the underlying immunologic mechanisms. This specific
biomarker also helps to distinguish the disease from other demyelinating
disorders. Here, we review the clinical and paraclinical features, immunological
properties and treatment options of the disease as reported in the literature.
Keywords :
neuromyelitis optica, aquaporin-4, children, optic neuritis, transverse myelitis.