TY - JOUR
T1 - The spectrum of neuromyelitis optica (NMO) in childhood
AU - Tillema, J. M.
AU - McKeon, A.
N1 - Funding Information:
The authors disclosed receipt of the following financial support for the research, authorship and/or publication of this article: Dr McKeon receives research support from the Guthy-Jackson Charitable Foundation.
PY - 2012/11
Y1 - 2012/11
N2 - The evaluation of inflammatory central nervous system disorders in childhood with predominant involvement of the optic nerves and spinal cord has been greatly enhanced over the last decade with identification of a group of disorders unified by the detection of neuromyelitis optica (NMO)-IgG, an antibody targeting the central nervous system-predominant water channel aquaporin-4. Clinical syndromes are predominated by the relapsing form of NMO but also include encephalopathic variants that can mimic acute disseminated encephalomyelitis. Maintenance immunotherapy is used to prevent relapses in NMO-IgG-seropositive patients. In contrast, NMO-IgG-seronegative children with NMO more commonly have a monophasic course (simultaneous occurrence of optic neuritis and transverse myelitis) and do not require remission-maintaining immunotherapy, but close surveillance is advised. Current clinical, pathological, and pathogenetic knowledge is reviewed with a focus on clinical presentation, neuroimaging findings, serological investigations, and treatment of children with disorders within the spectrum of central nervous system aquaporin-4 autoimmunity.
AB - The evaluation of inflammatory central nervous system disorders in childhood with predominant involvement of the optic nerves and spinal cord has been greatly enhanced over the last decade with identification of a group of disorders unified by the detection of neuromyelitis optica (NMO)-IgG, an antibody targeting the central nervous system-predominant water channel aquaporin-4. Clinical syndromes are predominated by the relapsing form of NMO but also include encephalopathic variants that can mimic acute disseminated encephalomyelitis. Maintenance immunotherapy is used to prevent relapses in NMO-IgG-seropositive patients. In contrast, NMO-IgG-seronegative children with NMO more commonly have a monophasic course (simultaneous occurrence of optic neuritis and transverse myelitis) and do not require remission-maintaining immunotherapy, but close surveillance is advised. Current clinical, pathological, and pathogenetic knowledge is reviewed with a focus on clinical presentation, neuroimaging findings, serological investigations, and treatment of children with disorders within the spectrum of central nervous system aquaporin-4 autoimmunity.
KW - aquaporin-4
KW - children
KW - neuromyelitis optica
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U2 - 10.1177/0883073812451495
DO - 10.1177/0883073812451495
M3 - Review article
C2 - 22859697
AN - SCOPUS:84867737343
SN - 0883-0738
VL - 27
SP - 1437
EP - 1447
JO - Journal of child neurology
JF - Journal of child neurology
IS - 11
ER -