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Complexity and wide range of neuromyelitis optica spectrum disorders: more than typical manifestations

Authors Han J, Yang M, Zhu J, Jin T

Received 27 July 2017

Accepted for publication 21 September 2017

Published 20 October 2017 Volume 2017:13 Pages 2653—2660


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Roger Pinder

Jinming Han,1 Meng-ge Yang,1 Jie Zhu,1,2 Tao Jin1

1Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China; 2Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden

Abstract: Neuromyelitis optica (NMO), considered to be mediated by autoantibodies, often cause severely disabling disorders of the central nervous system, and predominantly cause optic nerve damage and longitudinally extensive transverse myelitis. Remarkable progress has been made in deciphering NMO pathogenesis during the past decade. In 2015, the International Panel for NMO Diagnosis proposed the unifying term “NMO spectrum disorders” (NMOSD) and the updated NMOSD criteria reflects a wide range of disease and maintains reasonable specificity. Moreover, cumulative findings have indicated that NMOSD are frequently associated with multiple autoimmune diseases, thereby presenting complex clinical symptoms that make this disease more difficult to recognize. Notably, most neurologists do not heed these symptoms or comorbid conditions in patients with NMOSD. Whereas previous reviews have focused on pathogenesis, treatment, and prognosis in NMOSD, we summarize the present knowledge with particular emphasis on atypical manifestations and autoimmune comorbidities in patients with NMOSD. Furthermore, we emphasized the identification of these atypical characteristics to enable a broader and better understanding of NMOSD, and improve early accurate diagnosis and therapeutic decision making.

Keywords: neuromyelitis optica spectrum disorders, anti-aquaporin-4 antibody, comorbid conditions, atypical manifestations

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