Neurol Neuroimmunol Neuroinflamm 2015 Aug 23;2(4):e134. Epub 2015 Jul 23.
Stanford University (E.M., M.H.H.), Stanford, CA; Johns Hopkins University (M.L.), Baltimore, MD; University of Oxford (P.J.W.), UK; Tohoku University (D.K.S.), Sendai, Japan; University of São Paulo (D.K.S.), Brazil; University of Colorado (J.L.B.), Denver; Mt. Sinai University (G.R.J.), New York, NY; Thomas Jefferson University (D.C.H.), Philadelphia, PA; IDIBAPS (A.S.), Barcelona, Spain; Montreal Neurological Institute and Hospital (A.B.-O.), McGill University, Montreal, Quebec, Canada; Research Institute and Hospital of National Cancer Center (H.J.K.), Goyang, Korea; KS Hegde Medical Academy (L.P.), Nitte University, Mangalore, India; Oxford University Hospital (M.I.L.), Oxford, UK; University of Southern Denmark (N.A.), Odense; Vejle Hospital (N.A.), Denmark; University Hospital (N.K.), Marrakech, Morocco; MS Center (R.H.), Erasmus MC University Medical Center, Rotterdam, the Netherlands; Service de Neurologie A (R.M.), Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Bron, France; Molecular Neuroimmunology (S.J.), Department of Neurology, University Hospital Heidelberg, Germany; Tandem Labs (J.M.), San Diego, CA; University of Michigan Medical School (T.J.S.), Ann Arbor, MI; and David Geffen School of Medicine (M.R.Y.), University of California, Los Angeles.
Neuromyelitis optica (NMO) (and NMO spectrum disorder) is an autoimmune inflammatory disease of the CNS primarily affecting spinal cord and optic nerves. Reliable and sensitive biomarkers for onset, relapse, and progression in NMO are urgently needed because of the heterogeneous clinical presentation, severity of neurologic disability following relapses, and variability of therapeutic response. Detecting aquaporin-4 (AQP4) antibodies (AQP4-IgG or NMO-IgG) in serum supports the diagnosis of seropositive NMO. However, whether AQP4-IgG levels correlate with disease activity, severity, response to therapy, or long-term outcomes is unclear. Moreover, biomarkers for patients with seronegative NMO have yet to be defined and validated. Collaborative international studies hold great promise for establishing and validating biomarkers that are useful in therapeutic trials and clinical management. In this review, we discuss known and potential biomarkers for NMO.