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Review

Current and emerging therapies for

neuromyelitis optica

Cong Zhao, Hong-Zeng Li, Ya-Nan Bai, Zhu-Yi Li, Jun Guo

Department of Neurology, Tangdu Hospital, The Fourth Military Medical University, Xi’an 710038, Shaanxi, China.
A B S T R AC T
Neuromyelitis optica (NMO) is an autoimmune demyelinating disease that mainly affects the optic nerve and spinal cord, potentially
resulting in blindness and paralysis. Once thought to be a clinical variant of multiple sclerosis, NMO is currently considered as a different
disease with its own features due to the identification of a specific autoantibody against aquaporin 4. Given the high risk of disability,
treatment should be launched once the diagnosis is established. Evidence from clinical practice showed that traditional immunosuppressive
agents affecting the function of T and B cells could attenuate disease exacerbation. Recently, with better understanding pathogenesis of
NMO, increasing bodies of novel therapies and therapeutic targets have been discovered. In this review, the authors discuss the current
strategies of treating NMO in details and briefly introduce the potential therapies in future.

Key words: Neuromyelitis optica; aquaporin 4; therapies

INTRODUCTION systemic lupus erythematosus, Sjogren’s syndrome,
[7]
suggesting these patients may have a genetic tendency
Neuromyelitis optica (NMO) is an inflammatory to autoimmune.
demyelinating disease of the central nervous system
(CNS) characterized by recurrent optic neuritis and AQP4-IgG, a sero-biomarker of NMO, distinguishes
transverse myelitis. A relapsing-remitting clinical NMO from other demyelinating disorders and was
course was observed in the most patients. Historically, incorporated into the 2006 diagnostic criteria of
[8]
NMO was thought to be a variant of multiple sclerosis NMO, broadening the spectrum of NMO. In 2007,
(MS). Given its distinct clinical features, radiological neuromyelitis optica spectrum disorder (NMOSD) was
changes and the identification of the autoantibody termed to encompass patients who have detectable
targeted to aquaporin 4 (AQP4), it is now believed to serum AQP4-IgG but do not fully meet the diagnostic
[1]
criteria (e.g. first-atack LETM, typical NMO with brain
be a different disease with its own diagnostic criteria, lesions, NMO with other autoimmune diseases).
[9]
prognosis and treatment.
In 2015, the international panel for NMO diagnosis
unified the term of NMO and NMOSD, and developed
The epidemiology information of NMO is limited
because of its rarity and commonly confusing with MS. diagnostic criteria for seropositive and seronegative
[10]
NMOSD, respectively.
Estimated incidence and prevalence of NMO ranges
from 0.05-0.4 and 0.1-4.4 per 100,000 respectively. [2-5] The pathogenesis of NMO still remains ambiguous.
It has a preference in non-Caucasian women. The It is currently considered that AQP4-IgG is of
[6]
median age of disease onset is between 34-43 years, great importance in triggering NMO. AQP4 is a
while children and elder people are also affected. transmembrane protein expressed by astrocytes
[7]
Unlike MS, patients with NMO are more possibly and controls the flow of water in cells. Upon the
[11]
associated with other autoimmune diseases, like penetration through the blood brain barrier (BBB) and
binding with AQP4 on perivascular astrocyte end feet,
Corresponding Author: Dr. Jun Guo, Department of AQP4-IgG activates the classical complement pathway
Neurology, Tangdu Hospital, The Fourth Military Medical
University, Xi’an 710038, Shaanxi, China.
Email: guojun_81@163.com This is an open access article distributed under the terms of the Creative
Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows
others to remix, tweak, and build upon the work non-commercially, as long as the
Access this article online author is credited and the new creations are licensed under the identical terms.
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Website: For reprints contact: service@oaepublish.com
http://www.nnjournal.net
Cite this article as: Zhao C, Li HZ, Bai YN, Li ZY, Guo J. Current
and emerging therapies for neuromyelitis optica. Neuroimmunol
Neuroinflammation 2016;3:127-32.
DOI: 10.20517/2347-8659.2016.11
Received: 03-03-2016; Accepted: 13-04-2016

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