Conference Coverage

Long-Term Outcomes of Neuromyelitis Optica

The outlook for patients with NMO has improved since the discovery of AQP4-IgG.


PARIS—Among patients with neuromyelitis optica (NMO), early disability predicts late disability, consistent with favorable effects of early treatment on disability, according to a literature review presented at the Seventh Joint ECTRIMS–ACTRIMS Meeting. Additionally, outcomes are worse in nonwhite patients and Hispanic patients. Visual outcomes are worse in young-onset NMO, and motor outcomes are worse in late-onset NMO.

Zahra Nasr

Noting that there is limited literature regarding long-term outcomes in NMO spectrum disorder (NMOSD) since the discovery of aquaporin-4 immunoglobulin G (AQP4–IgG), Zahra Nasr, a medical student at Isfahan University of Medical Sciences in Isfahan, Iran, and colleagues sought to perform a systematic literature review on long-term outcomes in NMOSD in the era of AQP4-IgG.

The researchers conducted a database search that included studies in Cochrane Collaboration Database, PubMed, SCOPUS, Web of Knowledge, and Embase through April 2017. They used the search terms “neuromyelitis optica” or “Devic’s disease,” and “clinical features,” “outcome,” “natural history,” “prognosis,” “mortality,” “morbidity,” “incidence,” “prevalence,” “epidemiology,” and “demography.” They included in their analysis English language studies that used 1999, 2006, or 2015 Wingerchuk criteria and reported AQP4-IgG status.

Twenty percent to 30% of patients had residual motor and visual disability after the initial attack; early disability was positively associated with long-term disability. After five to six years, 11% to 18% of individuals had visual acuity of 20/200 or less in at least one eye, and 7% to 23% were wheelchair confined.

Nonwhite patients and Hispanic patients had higher relapse rates and worse outcomes. Younger patients and men had worse visual outcomes, whereas older patients had poor motor outcomes. In addition, long-term immunosuppressive treatment reduced attack-related disability. AQP4-IgG serostatus was not associated with outcome.

Survival improved in contemporary studies (91% to 98% survival after five years), compared with survival reported prior to the discovery of AQP4-IgG (68% to 75% survival). A higher attack frequency during the first two years, older age at onset, lack of recovery from first attack, blindness, and history of other autoimmune disease were associated with higher mortality rates, but race, gender, and type of attack at onset were not.

The researchers concluded that contemporary studies report more favorable outcomes than pre-AQP4-IgG series.

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